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SURGICAL

MANAGEMENT
OF STRABISMUS

EUGENE M. HELVESTON, M.D.

Emeritus Professor of Ophthalmology


Indiana University School of Medicine
Department of Ophthalmology
Section of Pediatric Ophthalmology and Strabismus
Indianapolis, Indiana

FIFTH EDITION
With 852 illustrations
With love and gratitude

to my wife

Barbara
Preface to the fifth edition

The 33 years since the publication of the Atlas of around the world. These patients' consultations were
Strabismus Surgery has seen a steady growth in the made possible by ORBIS Telemedicine, Cyber-Sight
number of ophthalmologists specializing in the diag- that is supported by the server at Flight Safety in New
nosis and treatment of strabismus. Membership in the York and provided through the generosity of Mr.
American Association for Pediatric Ophthalmology Albert Ueltschi. By viewing these images and read-
and Strabismus has grown to more than 500 mem- ing the discussion that accompanies each, it is my
bers and the International Strabismological hope that the reader will by critical analysis along
Association boasts 300 members in 25 countries. with comparison of the patients seen in his or her clin-
Several journals deal exclusively or largely with the ic hone skills for a more effective personal approach
subject of strabismus and several high quality texts to the whole patient leading to better outcome.
have been published dealing with strabismus diagno- This book represents the 'full circle' in that it is
sis and management. Moreover, an explosion in the based on experience during a professional lifetime in
field of information technology has brought people the care of patients with strabismus. It is my hope
together and facilitated the distribution of information that what I have learned and then shared here will be
in a way not dreamed of when the first edition of this of use to those who follow.
book was written. As with any effort like this there are many
This fifth edition is renamed Surgical deserving people to thank. First among these is Mrs.
Management of Strabismus reflecting the fact that the Lynda Smallwood who more than any person is
major emphasis has shifted from a description of sur- responsible for this book being completed. She not
gical procedures to a discussion of why and how only typed the manuscript, but also assembled and
these procedures are used to treat the patient with formatted the entire book making it ready for the
strabismus. There have been relatively few substan- printer by simply submitting it in electronic format
tive changes in surgical technique. It is how these ready for duplication. Truly a monumental task!
techniques are applied including the 'what' and 'when' Others who worked hard and effectively are Leslie
which make up the bulk of this book. Abrams, Michelle Harmon, Gwen Kopecky, Sharon
A new concept that has influenced surgical man- Teal who so ably carried on the art work begun by
agement during the last decade is that of the pulleys Craig Gosling, and Ann Hammer who read the first
associated with the horizontal rectus muscles. Study three chapters. I am grateful to the many Cyber-Sight
of these structures made possible in part by new partners who submitted hundreds of cases, and to my
imaging techniques, but more importantly by meticu- fellow Cyber-Sight strabismus mentors, especially
lous study of anatomic specimens, has helped make Dan Neely who faithfully and expertly responded
the management of 'A' and 'V' more logical and effec- when called on. I thank all of my teachers, especial-
tive. ly Gunter von Noorden my mentor beginning in 1966
Included in this book are 135 strabismus cases and my friend for life. Most of all, I thank my wife
managed by me either in the clinic or by means of Barbara for her unselfish and unfailing support, and
consultation carried out by me and other mentors on my two daughters both of whom had the good sense
patients sent by dozens of partners from countries to marry outstanding strabismologists.

Eugene M. Helveston

i
From the

Preface to the first edition

There have been several excellent texts on stra- the vista of strabismus surgery.
bismus including strabismus surgery in the past few For these reasons it seems appropriate at this
years, but developments have moved rapidly. Recent time to compile an up-to-date atlas of strabismus sur-
advances in technique have greatly expanded the gery. This atlas employs schematic drawings
options available to the strabismus surgeon. More designed to illustrate at each step only that anatomy
accurate diagnostic tests leading to a better under- significant to the step shown for easier orientation of
standing of the pathophysiology of strabismus and the reader. Procedures that I have found useful have
amblyopia have convinced some surgeons of the need been given emphasis; those that are controversial or
for surgery in infants as young as 5 months of age. that I have not found to be particularly helpful have
Improved anesthesia and an increasing boldness on been omitted. Some "favorite technique" may be
the part of the strabismus surgeon have led to outpa- omitted simply because I prefer an alternative choice;
tient extraocular muscle surgery in some instances those that I think should be avoided will be clearly
without patch and without ointment or drops. The labeled so.
limbal and cul-de-sac (or fornix) extraocular muscle No attempt will be made to give a set of surgical
exposure techniques have largely superceded the recipes that will result in a predetermined amount of
transconjunctival incision in the interpalpebral space straightening. Instead, general concepts leading to a
among younger surgeons. The retinal surgeon has philosophy for strabismus surgery will be presented.
opened new dimensions in the degree to which sub- My intent is that this atlas will be of help to the prac-
Tenons space may be explored. ticing strabismus surgeon and the resident in ophthal-
There have been several excellent texts on stra- mology by bringing together in one volume many
bismus including strabismus surgery in the past few techniques from a variety of sources for quick and
years, but developments have moved rapidly. Recent easy reference.
advances in technique have greatly expanded the Several people who assisted significantly in their
options available to the strabismus surgeon. More own way to make this atlas possible deserve my sin-
accurate diagnostic tests leading to a better under- cere thanks. Dr. Gunter K. von Noorden, teacher,
standing of the pathophysiology of strabismus and critic, and friend, introduced me to strabismus and to
amblyopia have convinced some surgeons of the need the pursuit of academic ophthalmology. Craig
for surgery in infants as young as 5 months of age. Gosling worked with industry and imagination on the
Improved anesthesia and an increasing boldness on illustrations, the heart of any atlas. My residents and
the part of the strabismus surgeon have led to outpa- many of my colleagues, in particular Drs. Marshall
tient extraocular muscle surgery in some instances M. Parks and Phillip Knapp, provided both stimulus
without patch and without ointment or drops. and direction.
New sutures, adhesives, muscle sleeves, and
implantation materials have proved useful innova-
tions. Globe fixation sutures, conjunctival recession
and relaxation procedures, forced duction and active
forced generation tests, as well as topical anesthesia
for extraocular muscle surgery, have greatly enlarged Eugene M. Helveston

iii
Contents

Section 1

1 History of strabismus surgery, 1 Anesthesia for strabismus surgery, 67


Preparation of the patient in the operating room, 70
Layout of the operating room and anesthetic
2 Surgical anatomy, 15 apparatus, 72
Patient monitoring, 73
Overview, 15 Magnification in strabismus surgery, 74
Palpebral fissure size, 15 Wearing a mask, 74
Extraocular muscle size, 16 Outpatient surgery: the day of surgery, 75
Pulleys, 18 Postoperative care of the patient, 76
Palpebral fissure shape, 21
Epicanthal folds, 22
Conjunctiva, 23 Section 2
Tenons capsule, 24
Surgical anatomy of the rectus muscles, 28
Characteristics of the extraocular muscles, 31 4 Work-up of the strabismus patient, 79
Motor physiology, 31 Design of the surgical procedure, 79
Underaction and overaction, 34 Step 1: Patient evaluation, 81
Surgical anatomy of the inferior oblique, 35 Diagnostic considerations for strabismus with
Lockwoods ligament, 37 restrictions, 90
Superior oblique, 38 Differential intraocular pressure test, 98
Whitnalls ligament, 41 Step 2: Results to be expected from surgery, 108
Trochlea, 42 Summary of steps 1 & 2 in the design of strabismus
Anterior segment blood supply, 48 surgery, 117
Vortex veins, 51 Step 3: Guidelines for application of surgical
Orbit and extraocular muscle imaging, 52 options, 117
Growth of eye from birth through childhood, 54 Step 4: Surgical technique, 117
Sclera, 56 Step 5: Follow-up of the surgical patient, 120

3 Parasurgical procedures and 5 Diagnostic categories and


preparation, 59 classifications of strabismus, 123
Overview, 59 Congenital esotropia, 125
Physical examination, 60 Intermittent exotropia, 142
Consent for strabismus surgery, 60 Brown syndrome, 147
Instruments used in strabismus surgery, 63 Duane syndrome, 149
Sutures and needles for strabismus surgery, 64 Superior oblique palsy, 151

v
Contents

Section 3

6 Mechanics of surgery, 163 9 Surgery of the obliques, 215


Techniques of exposure, 163 Oblique muscle surgery, 215
Limbal incision, 171 Weakening the inferior oblique, 216
Incisions for exposing the obliques, 174 Strengthening the inferior oblique, 225
Obtaining improved exposure, 175 Strengthening the superior oblique, 235
Cuffed superior limbal incision, 176
10 Marginal myotomy: technique and
7 Recession of a rectus muscle, 177 indications, 241
Overview, 177 Historical review, 241
Excessive recession, 177 Quantifying the marginal myotomy, 242
Recessions measured from?, 178 Technique for a double 80% marginal myotomy,
Medial rectus recession, 179 244
Lateral rectus recession, 181 Indications for a marginal myotomy, 245
Superior rectus recession, 182 Other considerations, 248
Inferior rectus recession, 184
Rectus muscle recession technique, 186 11 Faden operation (posterior fixation
Variations in suture and needle placement, 188
suture), 247
Vertical displacement of the horizontal rectus, 190
Resection effect of suture placement posterior to the Posterior fixation suture (retroequatorial myopexy,
muscle hook, 192 faden operation), 247
Hang-back recession, 193 Placement of a posterior fixation suture after
Partial disinsertion, 194 detaching the muscle, 251
Recession of a rectus muscle with sparing of the Placement of a posterior fixation suture without
anterior arteries, 194 detaching the muscle, 251
Slanted recession, 196 "Reinforced" posterior fixation suture, 252
Y split of the lateral rectus, 197 Combined posterior fixation and recession, 252
Adjustable faden, 252

8 Resection of a rectus muscle, 199


Overview, 199 12 Adjustable sutures: techniques for
Horizontal rectus resection (medial and lateral rectus
restriction, 255
muscles), 199
Management of the intermuscular membrane in Overview, 255
horizontal rectus resection, 200 Technique for the adjustable suture, 255
Resection clamp technique for rectus muscle Tandem adjustable suture, 259
resection, 201
Adjustable suture considerations, 260
Double-arm suture technique for rectus muscle
Conjunctival recession, 261
resection, 205
Traction sutures, 263
Resection of the superior rectus, 206
Inferior rectus resection technique, 207
Tandem suture technique for rectus muscle 13 Muscle transposition procedures, 265
resection, 208
Overview, 265
Plication (tuck) of a rectus muscle, 210
A review of muscle transposition procedures, 265
Displacement of horizontal rectus muscles with
Rectus muscle transfer, 268
resection for A and V patterns, 211
Scleral augmented muscle-tendon transfer, 270
Vertical effect from horizontal rectus resection and
recession, 212 Knapp procedure, 272
Superior oblique tendon transfer, 273
Transposition for head tilt without oblique muscle
dysfunction, 274

vi
Contents

14 Botox (Botulinum A-toxin), 275


Overview, 275 Inclusion of the inferior oblique in the lateral
The drug, 275 rectus insertion, 471
Indications for Botox, 276 Muscle-tendon rupture, 472
Use of Botox in treatment of strabismus, 276 Hyphema, 472
Retrobulbar Botox for treatment of nystagmus, 277 Posterior chamber hemorrhage, 473
Botox for treatment of benign essential Motility disturbance after nonmotility procedures,
blepharospasm, 277 473
Technique of injection, 277 Postoperative Brown syndrome, 473
Botox for the treatment of blepharospasm, 281 Symblepharon, 474
Injection techniques for blepharospasm and facial Orbital hemorrhage, 475
spasm, 281 Orbital cellulitis, 476
Endophthalmitis, 476
Postoperative communication, 477
Section 4 Operation on the wrong muscle, 477
A homily, 477
Operation on the wrong patient, 477
15 Telemedicine: distance medicine, 285
Undesirable overcorrections and undercorrections,
Real time vs. store and forward, 285 478
Moving or still, 285
Image size, 285 Reading list, 479
Computer, 286
Start of Cyber-Sight, 286
Telemedicine consultation cases, 292 Index, 495

16 Strabismus case management, 359

17 Complications in strabismus surgery,


455
Complications of strabismus surgery?, 455
Criteria for success after strabismus surgery, 455
Informed consent, 457
Diplopia, 457
Reoperation, 458
Loss of vision, 458
Postoperative nausea and vomiting, 458
Acute, allergic suture reaction, 459
Chronic suture granuloma, 460
Reaction to synthetic absorbable suture, 460
Subconjunctival cysts, 460
Prolapse of Tenon's capsule, 462
Suture abscess, 463
Delle, 463
Lid fissure anomalies, 464
Ptosis of the upper lid, 465
Scleral perforation, 465
Slipped or lost muscle, 466
Anterior segment ischemia, 469
Persistent overaction of the inferior oblique
muscle, 470
Inferior oblique adherence syndrome, 470

vii
Section 1

Chapter 1: History of strabismus surgery

Chapter 2: Surgical anatomy

Chapter 3: Parasurgical procedures and preparation


1
History of strabismus surgery

Strabismus, recognized from the earliest times In 1816 Dulspech, an orthopedic surgeon, performed
because the eyes are readily observable, has changed tenotomy of the Achilles tendon as treatment for club
little in form throughout the years. However, its caus- foot with a technique not too different from strabis-
es are now well understood, and its treatment under- mus surgery. Gibson experimented with extraocular
goes regular revision and refinement. The condition,
at first, was considered to be a visitation of an evil
spirit and incurable. The earliest physicians suggest-
ed treatment with the only available methods--
potions, purification, and diet. In the seventh centu-
ry, masks designed to redirect the visual axes were
described. Curiously, variations of this type of treat-
ment are still advocated by some today in the form of
sector occlusion, despite 13 centuries of failure!
The practice of strabismus surgery began inaus-
piciously in 1739 with the efforts of John Taylor
(Figure 1). He is reported to have had ...a consider-
able amount of sense..., according to Stewart Duke-
Elder, who said Taylor undoubtedly recognized that
strabismus was a muscle abnormality that could be
treated by dividing the extraocular muscles.*
However, Taylors surgery was not successful. On
the contrary, Taylor is said to have been a showman
who may have only snipped the conjunctiva, patched
one eye, and left town before the results could be
assessed. By patching the unoperated eye and having
the operated eye take up fixation, Taylors procedure
would have given the appearance of being successful
since the operated eye would appear to have been
straightened. John Taylors position in history
appears to be one of ridicule rather than honor.
After Taylor, several surgeons contributed to
the body of knowledge that led to successful, scien-
tifically founded strabismus surgery. In 1752, Figure 1
Eschenbach made the comment each oculist dreams Abundant showmanship and fast carriage rather than
from time to time that it may be possible to dissect surgical skills were outstanding attributes of the first
completely or partially the offending rectus muscle. strabismus surgeon John Taylor (1703-1772), an itinerant
healer who is depicted in this eighteenth century drawing.

*Duke-Elder S and Wybar K: System of ophthalmology, Vol 6, Ocular motility and strabismus, St Louis, 1973, The CV Mosby Co.
von Noorden GK (ed): History of Strabismology, Belgium 2002, J. P. Wayenborgh

1
Chapter 1

muscle transection for treatment of esotropia in 1818


but did not report this until 1841. White, in 1827,
made similar attempts without success. Stromeyer
performed extraocular muscle tenectomy on a cadav-
er in 1838, and a year later, Dieffenbach performed
successful myotomy of the medial rectus on a 7-year-
old boy with esotropia. His priority was contested by
Cunier, but the honor has been granted to
Dieffenbach. The year 1839 is generally considered
to be the beginning of modern strabismus surgery.
In the nearly century and a half since its begin-
ning with Dieffenbach, strabismus surgery has under-
gone many refinements, but the basic principles have
changed little. The orthopedic principles that were
adhered to at the beginning of strabismus surgery
remain as the primary guiding factors today. To these
have been added new knowledge of the central nerv-
ous system influences and psycho-physical factors
related to strabismus. Understanding of neural mech-
anisms combined with knowledge and appreciation of
both the dynamic muscle forces and the physiologic
and pathologic restrictive aspects of ocular motility
make strabismus surgery a science. Improved diag-
nostic techniques, such as passive duction testing, Figure 2
interpretation of differential intraocular pressure, dif- Taylor may have only snipped the conjunctiva, patched the
ferential saccadic velocity measurement, electroocu- eye, and then left town. He is said to have operated twice
logram testing of nystagmus, and generated muscle on Bach, the musician of Leipzig, who later became blind.
The antics of Taylor may have been responsible for a 100-
force testing, have enabled more accurate diagnosis year delay in the establishment of strabismus surgery as
leading to more accurate surgery. part of the surgeons armamentarium.
Improved imaging techniques, especially those
using magnetic resonance imaging (MRI), have made
possible the noninvasive morphologic study of
extraocular muscles. Technical improvements in
anesthesia, sutures and needles, implanted materials, A
antibiotics, corticosteroids, and, most recently,
injectable toxins have broadened the scope of strabis-
mus surgery. Recent studies of the origins of the most
common strabismus, essential infantile or congenital
esotropia, have promoted earlier surgery. Strabismus
surgery, frequently done as an office procedure in its
early years, is now once again done on an outpatient
basis.
Strabismus surgery began with weakening pro-
cedures of the medial rectus. These procedures were B
first performed by transecting the muscle and often
produced disastrous results (Figure 2). Shifting sur-
gery from the belly of the muscle to the tendon
reduced the number of overcorrections (Figure 3).
Tendon weakening or lengthening, which avoided
cutting across the full width of the tendon, was sub-
sequently carried out using a variety of ingenious
techniques. The earliest of these were performed Figure 3
A Myectomy of the medial rectus for esotropia, as
without placing a needle and suture through the ten- performed by Dieffenbach in 1839, often produced a
don. Instead, one or more incisions were made in the large exotropia and unsightly medial displacement of the
muscle or tendon, usually at right angles to the long caruncle. The so-called cure may have been only a
axis of the muscle and crossing the midline. Later, slight improvement over the malady.
B Simple tenotomy carried out nearer the insertion
tendon lengthening procedures used sutures. (Figure reduced the number and severity of overcorrections and
4 & 5). lessened caruncle displacement.

2
History of strabismus surgery

Figure 4
A wide variety of tendon lengthening procedures provided a safeguard against overcorrection and could also be carried out
without sutures, The simple Z tenotomy (Blaskovics, 1912) is still used today for weakening an already maximally recessed
muscle (Chapter 4).
A OConnor E Abadie
B Bishop-Harmon F Verhoeff
C Blaskovics G Terrien
D von Graefe
Some modern surgeons are reporting satisfactory clinical results from tenotomy which does not cut across all fibers, but
more or less nicks the muscle. Biglan reports success treating small vertical deviations by doing incomplete tenotomy of
the superior rectus.

3
Chapter 1

Figure 5
The use of sutures added to the complexity and safety, if not the effectiveness, of tenotomy. Again, the principle was that
a lengthened tendon-muscle would result in reduced muscle pull and therefore produce a change in alignment, shifting the
globe away from the weakened muscle.

4
History of strabismus surgery

A B

Figure 6
A Sutures were also used to reapproximate myotomized B Prince (1887) was credited with describing recession
muscles to avoid producing more than the intended with the tendon actually sutured to the sclera. This
result. The sutures were not placed in the sclera. They procedure was the forerunner of more accurate and
were placed only in the cut ends of the muscle and reproducible surgery.
tendon. The initial use of sutures apparently was for C Worth (1904) described resection using sutures. This
overcorrected and not primary cases. muscle shortening was accomplished with or without
recession of the antagonist muscle.

Late in the nineteenth century, measured reces- advances in anesthesia. A glass of wine and a good
sion with reattachment of the muscle to the sclera was lunch followed by a head lock and a quick surgeon
carried out. Tendon and muscle tucking or plication; were early anesthesia. This was replaced by ponto-
muscle advancement procedures; and, later, resection caine, cocaine, and ether for general anesthesia, and
and advancement procedures were also performed later, a wide variety of modern agents were used.
(Figure 6). Supporting modern anesthetic agents are the exten-
Surgery of the oblique muscles began when sive and detailed monitoring devices that provide
inferior oblique weakening was carried out to treat instantaneous, real-time recordings of temperature,
myopia and asthenopia, not surprisingly without suc- respiration, electrocardiographic data, and oxygen
cess. The superior oblique muscle was considered by saturation.
some noli me tangere, loosely translated today as off Highlights of the history of strabismus sur-
limits, in the early twentieth century. However, all gery from its earliest beginnings in 1739 to the pres-
types of oblique surgery had been described by the ent are depicted in the following drawings. They
middle part of the twentieth century. Fink, in 1951, describe a wide variety of manipulations that sur-
said up to a few years ago, all corrective surgical geons employed to straighten the eyes by altering the
measures for the obliques were not considered feasi- state of the extraocular muscle (Figures 7-24). The
ble because of anatomical difficulty.* statement has been made, Those who do not learn
Along with oblique muscle surgery and vertical from history are destined to repeat it. Thus, some
rectus muscle recession and resection, extraocular surgical procedures have a tendency to be rediscov-
muscle transfer procedures were used for strabismus ered every generation or so by surgeons who may
caused by muscle paralysis. Although, at first, the have overlooked an earlier description. Nonetheless,
improved alignment after eye muscle transfer proce- strabismus surgery has followed a steady progression,
dures was thought to be due to postoperative neuro- with improvement through innovations in the surgical
logic reorientation, it became apparent from elec- technique, combined with better instruments, more
tromyographic studies that the effect of an extraocu- reliable sutures, safer anesthesia, and a better appre-
lar muscle transfer procedure was mediated through ciation on the part of the surgeon of neural, sensory,
mechanical factors. and mechanical factors.
Technical advances in strabismus surgery have
occurred along with, and in some ways because of,

*Fink WH: Surgery of the oblique muscles of the eye, St Louis, 1951, The CV Mosby Co.

5
Chapter 1

Figure 7
Most early recession procedures involved sutures entering and exiting the conjunctiva for added security and to facilitate
removal of silk sutures. Tenons capsule and the muscle capsule were incorporated in recessions and resections. The
muscle is cut between bites of the preplaced suture to avoid loss of the disconnected proximal muscle end. Externally
placed buttons or bolsters added additional support to the recessed muscle, facilitated suture adjustment, and made suture
removal easier.

Figure 8
Jamesons technique for recession included several sutures anchoring the muscle to the sclera. The external sutures could
be removed and loosened or tightened postoperatively if a significant overcorrection or undercorrection was produced.

6
History of strabismus surgery

Figure 9
A Early needles were round; had eyes for threading the B Jameson admonished surgeons to keep the needle in
suture; and in most cases, had a wire diameter greater view while passing it through the sclera, to avoid entering
than the thickness of the sclera. These factors made the the globe and creating what he described as panoph-
ophthalmic needle a formidable weapon. thalmia.

Figure 10
Peters whip stitch for securing the cut end of the muscle during recession of a rectus muscle is a sound technique that is
still used today.

7
Chapter 1

A B

Figure 11
A A simple tuck for muscle-tendon shortening (strengthen- B When performed near the limbus, tucking tended to pro-
ing) avoided the need for placing a needle through the duce unsightly bulk that could be cosmetically disfiguring.
sclera and produced a shortened muscle without remov-
ing a piece.

Figure 12
Pragnen used gold buttons as bolsters to eliminate cheese wiring of the externalized suture when carrying out resection of
a rectus muscle. This technique allowed postoperative adjustment of the length of the muscle.

Figure 13
Peters modification of the Bishop tucking instrument was
one of several elegant instruments that were designed to
facilitate the tucking procedure. Tucking of the extraocular
muscles was an early favorite muscle shortening strength-
ening procedure.

8
History of strabismus surgery

Figure 14
An ingenious technique, the OConnor cinch, produced minimal shortening of
a rectus muscle. A large, dull needle was used to weave a multiple-strand
suture through slips of the tendon or muscle. The greater the number of
threads, producing a bulkier suture and a greater diameter of thread, the
more shortening of the muscle is produced.

B
A

Figure 15
A Peter suggested fracturing the trochlea and then shifting B The lateral rectus was shifted for treatment of congenital
and attaching the superior oblique muscle to a point near absence of the inferior rectus.
the medial rectus insertion. This procedure was com-
bined with lateral rectus tenotomy or recession for treat-
ment of third cranial nerve palsy.

Figure 16
A transcutaneous approach to the superior oblique tendon
afforded exposure for tenectomy or tucking. Skin incisions
were commonly used for exposure of both superior and infe-
rior oblique muscles in the early twentieth century.

9
Chapter 1

A1 A2

B C

D E

Figure 17
A1 Wheeler exposed the inferior oblique muscle nasal to E Wheeler strengthened the superior oblique muscle by
the inferior rectus through a skin incision. advancing the central portion of the tendon. He recog-
A2 The inferior oblique muscle was then engaged with a nized that this procedure produced decreased elevation
in adduction. In addition, he recognized that shifting the
muscle hook.
tendon anteriorly produced intorsion and that posterior
B From that exposure the muscle could be weakened by fibers influenced depression of the globe, a concept later
myotomy. credited to Harada and Ito (Jpn J Ophthalmol 8:88,
C The muscle also could be shortened by a tuck. 1964).
D A curious inferior oblique strengthening procedure per-
formed by Wheeler was disinsertion of the muscle fol-
lowed by reinsertion of the cut end into the inferior
orbital rim periosteum.

10
History of strabismus surgery

Figure 18
Hughes and Bogart exposed the trochlea subperiosteally, freed it, and pushed it backward several millimeters to weaken
the superior oblique. This formidable procedure was accomplished with a transcutaneous approach. No reports of series
of patients having this procedure performed on them were described. It is unlikely that his procedure was done widely; it
indicates the imagination and ingenuity of the early strabismus surgeon. This procedure, when performed inadvertently
after use of the Lynch incision for exposure of the ethmoid sinus, can produce superior oblique underaction, creating the
clinical picture of superior oblique palsy which, in turn, results in diplopia for the unlucky patient.

Figure 19
White plicated the superior oblique tendon medial to the superior rectus using a tucking instrument. He advised placing the
tuck nearer the superior rectus and away from the pulley of the trochlea to avoid having the superior oblique tendon become
hung up in the trochlea.

Figure 20
McLean engaged the superior oblique tendon temporally and brought the redundant loop of tendon temporally. This technique
for tucking the superior oblique tendon was said to reduce the incidence of postoperative limitation of elevation in adduction
by avoiding the trochlea and by reducing the likelihood of adherence of the redundant loop of tendon to the superior rectus.
However, any superior oblique tuck will cause a Brown postoperatively unless the tuck is done in a loose tendon and will result
postoperatively in an equal and normal superior oblique traction test (see chapter 9).

11
Chapter 1

Figure 21
Based on a procedure originally suggested by McGuire, Fink described resection of
the superior oblique tendon after detaching the superior rectus muscle. Removal
of the superior rectus provided improved exposure of the superior oblique tendon.

Figure 22
Fink recessed the inferior oblique muscle using a recession localizer that had a 7 cm handle with 6 mm arms projecting at
90 degrees from each other and a 1 mm tip with a concavity to hold gentian violet for marking. The purpose of the instru-
ment was to allow for an accurate 8.0 mm recession of the inferior oblique muscle. By estimation the recession could be
reduced to 6.0 mm or increased to 10.0 mm.

Figure 23
Following the lead of Duane, White and
Brown weakened the inferior oblique by
disinsertion without reattachment, consider-
ing the procedure safe and effective.

12
History of strabismus surgery

A B1 B2

C D E

F G H

I J K

Figure 24
Several techniques have been described for extraocular muscle transfer. The underlying principle for each procedure,
except superior oblique tendon transfer, is to shift the pull of antagonist muscles to a point on the globe coinciding with the
insertion of the rectus muscle lying between. The procedures shown have been described by the following:

A Hummelsheim F Schillinger
B1 OConnor G Beren-Girard
B2 Modified OConnor H Jensen
I Uribe
C Wiener
J Knapp
D Peter
K Helveston*
E Hildreth
*done in the case of an absent medial rectus

13
Chapter 1

A limited number of manipulations can be per- in early literature dealing with strabismus surgery.
formed on an extraocular muscle. The muscle can be Modern strabismus surgery differs significantly from
shortened and then made to stretch, at first under ten- early strabismus surgery in its attention to detail in
sion, to the original preoperative distance between dealing with the orbital fascia and fat. Recent
origin and insertion while presumably being more description of the anatomy of the trochlea, the inferi-
effective; the muscle can be retroplaced with the or oblique, and especially of the muscle pulleys has
same length of muscle going between two points provided new insight for strabismus surgeons.
closer together than the original origin and insertion; These tissues are thought by some surgeons to
or a longer muscle connecting the original origin be as important as the muscle itself. This book will
and insertion can be created with myotomy or tenoto- emphasize the importance of technique in the belief
my. The insertion can be shifted to a new place on that the surgeon who exercises impeccable care when
the globe, retaining the same innervation but having dealing with all tissues encountered in strabismus sur-
an altered mechanical effect. gery will attain better results.
Recent innovations in strabismus surgery Some operations that have been suggested by
include change in the arc of contact produced by the older surgeons or a few contemporary surgeons are
Faden operation (retroequatorial myopexy or posteri- not mentioned even as an option in either the histori-
or fixation suture); denervation of the inferior oblique cal or current sections. These omissions indicate a
muscle; detachment of the rectus muscles with spar- bias, which I hope will not result in overlooking use-
ing of the anterior ciliary vessels; and toxin injection ful procedures. Only procedures of sufficient histori-
at the motor end-plate to weaken a muscles function cal interest or contemporary procedures that have
by interference with nerve transmission at the some rational basis will be described. When appro-
myoneural junction. Thermal disruption of selected priate, alternative techniques will be described.
eye muscle fibers has been described but has not yet The use of adhesives and plastic sleeves and
been proven successful. sheets or caps mentioned in previous editions now are
Early strabismus surgeons had an accurate mentioned here only as a part of the history of stra-
grasp of muscle function but little appreciation for bismus surgery. Like many of the procedures shown
orbital fascial anatomy. Numerous references to the in this chapter, these techniques sounded good, were
encountering of fat during strabismus surgery suggest tried, but now are relegated mostly to memory.
that even the best surgeons found orbital fat on a rou- For a more complete review of the history of
tine basis. Except for referring to cutting across the strabismology, the reader is advised to consult The
conjunctiva, Tenons capsule, and the muscle capsule, History of Strabismology edited by Gunter von
scarcely a comment on these fascial tissues is found Noorden (J. P. Wayenborgh, Belgium, 2002).

14
2
Surgical anatomy

Overview
The successful strabismus surgeon has a clear extraocular muscle surgery. The orbital fat must be
understanding of the anatomy of the extraocular mus- recognized, respected, and left undisturbed.
cles which move the eyes, and also of the fascia, fat, Lockwood's ligament and associated lower lid retrac-
nerves, and the blood supply of the eye and orbit. tors providing support to structures that influence the
Although the extraocular muscles, especially that part lower lid position and to the inferior oblique and infe-
anterior to the equator, are the focus of strabismus rior rectus muscles must be dealt with properly to
surgery, structures such as the conjunctiva, anterior avoid lower lid ptosis. Whitnall's ligament associated
Tenon's capsule, posterior Tenon's capsule (intermus- with the levator palpebri, while not ordinarily
cular membrane and muscle sheaths), and the newly- encountered in the course of strabismus surgery, plays
described pulleys influence both movement and an important part in surgery of the upper lid. The vor-
alignment of the eyes. The sum of these structures, tex veins, although often observed in contrast to
including those observed during the course of surgery orbital fat which is not, should not be disturbed.
and others never seen, work in concert actively and Much of the new information about the anato-
passively to support, guide, restrict, or modify move- my and physiology of the structures of the orbit
ment of the eyes both physiologically in health and comes from laboratory studies using ultrasonography,
pathologically in the case of strabismus. computerized tomography (CT), magnetic resonance
The surgeon must be concerned with the imaging (MRI), and histochemical techniques. Use of
mechanics of access to the operative site, first some of these in clinical practice can also aid in stra-
between the lids and then through the conjunctiva and bismus diagnosis and in the design and execution of
Tenon's capsule. A proper start is required if the sur- successful extraocular muscle surgery.
geon expects a successful conclusion. The location, The following material describing surgical
as well as the blood supply, innervation, and action of anatomy and functional physiology is intended to
each of the extraocular muscles, must be known, provide practical information for the strabismus sur-
including the contribution of each muscle's intrinsic geon.
blood supply to the nutrition of the anterior segment.
Scleral thickness, which varies according to location, Palpebral fissure size
must be taken into account especially when placing a The dimensions of the palpebral opening
needle into the sclera. increase nearly 50% in width and 20% in height
The action of each extraocular muscle is influ- between infancy and adulthood. Configuration varies
enced by the location of its insertion on the globe and with a persons physical and racial characteristics
by the location of its pulley (or equivalent structure), (Figure 1). The size and shape of the palpebral open-
in particular, those pulleys associated with the medial ing should be considered at the outset of extraocular
and lateral rectus muscles. A thorough understanding muscle surgery. To start with, a lid speculum appro-
of these anatomic relationships forms the basis for a priate to the size of the palpebral opening should be
logical mechanical approach to surgery on the used (Figure 2). In addition, the surgeon should
extraocular muscles, including recession, resection, expect to encounter more difficulty with exposure
transposition, transection, plication, union, and and suture placement in medial rectus recession and
chemodenervation. In addition, orbital structures sur- also in patients with a small palpebral fissure or
rounding the globe and extraocular muscles pro- deeply-set eyes. However, measured recession can be
foundly affect the design, execution, and results of

15
Chapter 2

accomplished even with the smallest lid fissure open-


ing in an infant beginning at 4 months. Limited work-
ing area is not an adequate reason for doing a mar-
ginal myotomy as an initial weakening procedure of a
medial rectus muscle in congenital esotropia simply
because a marginal myotomy is thought to be easier
to accomplish than a measured recession. Extraocular
muscle surgery in an adult with deeply-set eyes and a
smaller than average palpebral opening can be more
difficult than such surgery in a 3- or 4-year-old child
Figure 1
with a normal or larger than normal palpebral open- The average adult palpebral opening is 28 mm long and 10
ing (Figure 3, 4). mm high.
Ptosis, pseudo ptosis, lid retraction, exophthal-
mos, and enophthalmos all represent lid and palpebral
fissure variations that will be encountered and must
be both understood and dealt with in the gamut of
strabismus management. These issues will be dealt
with in more detail during the discussion of clinical
examples.

Extraocular muscle size


Unlike the palpebral opening which differs sig-
nificantly according to age, the extraocular muscle
size, or at least width at insertion, is closer to constant
throughout life. A child with a tiny palpebral opening
is likely to have a medial rectus whose insertion
width is very close to the adult average measurement
of 10 mm. This means that the timing of strabismus
surgery is not determined by either the size of the
palpebral opening or of the extraocular muscles. Figure 2
Lieberman speculums, pediatric (top) and adult (bottom),
Globe size, on the other hand, is significant in the are ideal for strabismus surgery.
design of strabismus surgery. Comparing globe size
and extraocular muscle location, Swan and co-work-
ers pointed out that in newborns, the posterior part of
the globe is relatively smaller than the anterior part,
meaning that a recession of 3 mm could place the
medial rectus at the equator. This is important infor-
mation but not for strabismus surgery, which is not
indicated anyway in the newborn because of immatu-
rity of the binocular system. The globe in the 4-
month-old, an age some consider the earliest appro-
priate for strabismus surgery, is 19.5 mm in axial
length. This is sufficient size to allow an appropriate Figure 3
recession of the medial rectus in congenital esotropia. An average 18-month-old child has a palpebral opening 20
For example, a recession of 9.5 mm measured from mm long and 8.5 mm high.
the limbus places the new insertion of the medial rec-
tus at the equator in a 4-month-old with an axial
length of 19.5 mm (Figure 5). The insertion of the
medial rectus in an infant can be closer than 5.5 mm
from the limbus. This means that a medial rectus
recession, if measured 9.5 mm from the limbus, will
be at least equal to a 4 mm recession, if measured
from a medial rectus insertion that was 5.5 mm from
the limbus. It could also be as large as 6 mm consid-
ering measurement from the insertion, if the medial
rectus inserts 3.5 mm from the limbus as it does in Figure 4
some infants. With continued growth of the globe A newborn has a palpebral opening measuring 18 mm long
and 8 mm high.

16
Surgical anatomy

taking place primarily in the posterior part, the rela- undercorrection of congenital esotropia occurred in
tive position of the medial rectus insertion will move nearly 50% of cases when the maximum for medial
anteriorly, and therefore safely, with maturity. rectus recession was on the order of 5.5 mm from the
The point of measuring from the limbus is that insertion as was the rule in the 1960's. This under-
this provides a safe technique for performing a larger correction rate reduced immediately to approximate-
recession of the medial rectus, especially in those ly 10% in a series when medial rectus recession was
cases with medial rectus insertions closer to the lim- measured from the limbus. This technique of reces-
bus (Figure 6). Measurement from the limbus begs sion measured from the limbus, allowing larger but
the question, "Is the significant factor in correction of safe recessions, is now joined by larger recession
esotropia the size of medial rectus recession or the measured from the insertion. These larger recessions,
new position of the muscle on the globe?" The some 7 mm or more, have been implicated in a high-
answer may be that both play a role. We do know that er overcorrection rate.

A B

Figure 5
A The medial rectus of a 4-month-old measures B The muscle has been recessed 9.5 mm from the limbus
approximately 10 mm at the insertion. using a limbal incision.

Figure 6
In a 4-month-old with an axial length of 19.5 mm, a recession 9.5 mm from the limbus places the new insertion of the
medial rectus approximately at the equator.

17
Chapter 2

Pulleys
Around 1990, Joseph Demer and associates
began study of the anatomy and actions of the A
extraocular muscles using high resolution magnetic
resonance imaging on clinical patients and normal
subjects aided in some cases by the use of paramag-
netic MRI contrast agents (Figure 7, 8). This work,
along with detailed histologic and histochemical
study of human and monkey orbital tissue in the lab-
oratory, led to the following summation by the C
authors in 2002. "The resulting reexamination of
EOM (extraocular muscle) anatomy and physiology
has been so revealing as to suggest a fundamental
paradigm shift having broad basic and clinical impli-
cations."
Demer and associates conclusions are summa-
rized as follows: B
1. Orbital structures called pulleys are associ-
ated with each of the rectus muscles and the SAGGITAL
inferior oblique.
2. The pulleys receive the contractile force of Figure 7
The pulley zone is roughly at the junction of the middle
the extraocular muscles and inflect the paths and posterior third of the globe, similar to Listings plane
of the muscles in a "qualitatively similar (see page 32).
manner to the inflection of the superior A Trochlea of the superior oblique - inflection of the
oblique (SO) tendon path by the trochlea" superior oblique.
B Lockwood ligament - the pulley of inferior rectus -- the
(Figure 9). functional origin of the inferior oblique (the functional
3. The paths of the extraocular muscles posteri- insertion of the inferior oblique after distal myectomy).
or to the pulleys (between the pulleys and the C The pulley of the horizontal recti.
annulus of Zinn) remain constant regardless
of the position of the globe. There is no side
slip of the rectus muscles, (except in the case
of an abnormality of the pulley).
4 . The functional origin of the extraocular mus-
cles is at their pulleys (Figure 10).
5. The orbital one-half of the extraocular mus-
cle fibers insert into the pulley and the bulbar
one-half of extraocular muscle fibers pass
forward to attach to the globe at the muscles
insertion (Figure 11).
6. Only that portion of the extraocular muscle
anterior to the pulleys moves in the direction
of the globes movement (Figure 12).
7. Upward displacement of the lateral rectus
pulleys and downward displacement of the
medial rectus pulleys are associated with A
pattern. (Figure 13) Downward displacement CORONAL
of the lateral rectus pulleys and upward dis-
placement of the medial rectus pulleys are Figure 8
associated with V pattern (Figure 14). A Trochlea
8. Pulleys made up of collagen, elastin, and B Confluence of superior oblique tendon and superior
rectus sheath
richly innervated smooth muscle are situated C Lockwoods ligament
in the orbit in the area previously called D Pulley of the horizontal recti
check ligaments. They are not readily distin- E Whitnalls ligament
guishable clinically and require special tech- F Levator palpebri
niques to be seen in the laboratory.
9. Several other pulley abnormalities could be
associated with strabismus entities including:

18
Surgical anatomy

Figure 9
The pulleys which inflect the paths of the muscle.
A Medial rectus pulley
B Lateral rectus pulley

B
D

Figure 10
The functional origin of the rectus muscles is at the pulleys.

Figure 12
The muscle - tendon anterior to the pulley
A Passes straight in primary position
B Courses upward in upgaze
C Courses downward in downgaze
D The direction of the muscle posterior to the pulley does
not change during up and downgaze

Global
Orbital

Figure 11
The orbital fibers insert into the pulleys of the horizontal
recti and the global fibers insert into sclera.

19
Chapter 2

Y pattern exotropia (pulley instability of


the superior rectus, inferior rectus and lateral
rectus); incomitant strabismus (abnormal
sideslip of rectus extraocular muscle paths in
certain gaze positions); and Brown syndrome
(downward shift of the lateral rectus pulley in
adduction or supraduction).
The strabismus surgeon should be aware of the
existence of pulleys and of their significance when
undertaking the management of strabismus.
Although the surgeon will neither observe nor manip-
ulate these structures except in special cases, they are
nonetheless an important factor in establishing a
proper diagnosis and in designing the best surgical
procedure in many cases. An appreciation of the func-
tion of the pulleys contributes to understanding the
behavior of the eye movements in the strabismus
patient as well as in the normal. Diagnosis, plan for
treatment, and assessment of the outcomes of treat-
ment of strabismus are enhanced by knowledge of the
Figure 13
extraocular muscle pulleys.
Some combination of:
Upward displacement of the lateral recti The reasons that the strabismus surgeon is not
Downward displacement leads to A pattern likely to see the pulleys are several. First, surgery of
the extraocular muscles is carried out beneath anteri-
or Tenon's capsule and in the plane of posterior
Tenon's capsule. It is done anterior to the origin of
anterior Tenon's capsule which itself is just anterior to
the location of the pulleys. Second, dissection car-
ried posterior to the origin of anterior Tenon's capsule
(where it can be seen fusing with the muscle sheath or
posterior Tenon's capsule) will expose extraconal fat,
which both complicates surgery and obscures the sur-
rounding anatomy, including the pulleys. Third,
although the pulleys are located in the orbital fat just
behind the insertion of anterior Tenon's capsule, they
are virtually impossible to identify for what they are.
This should be obvious since these structures escaped
detection for nearly 200 years, in spite of extensive
study of the contents of the human orbit carried out by
many competent investigators.
Prior to the studies of Demer and associates, the
closest anyone could come to identifying these struc-
tures was a description of the check ligaments of
the horizontal recti, Whitnall's ligament superiorly,
the trochlea, and Lockwood's ligament. By asking
the right questions and using advanced techniques for
Figure 14 imaging and histochemical analysis, along with
Some combination of: meticulous dissection and histologic study, Demer
Downward displacement of the lateral recti
Upward displacement of the medial recti leads to V and associates accomplished the difficult task of
pattern describing newly-recognized anatomy. But, as with
so many other discoveries in science, this new revela-
tion came after the earlier work of others which gave
hints of what would be. In this case, the observation
of Urrets-Zavalia, which called attention to the rela-
tionship of palpebral fissure configuration and verti-
cal incomitance, is certainly a precursor to the revela-
tion of pulley displacement. The long known associ-
ation of V pattern with the pronounced antimon-

20
Surgical anatomy

goloid fissures of Crouzon and the individuals with


true mongoloid fissures who demonstrate an A pat-
tern also provided clues. Limon in Mexico was par-
ticularly instrumental in correlating orbital anatomy
with a variety of strabismus patterns which were no
doubt influenced by the as yet undiscovered pulleys.

Palpebral fissure shape


The palpebral fissure may be level, mongoloid,
or antimongoloid, depending on the relative positions
of the medial and lateral canthi. If the outer canthus is
higher than the inner canthus, a mongoloid palpebral
slant exists (Figure 15). If the outer canthus is lower
than the inner canthus, an antimongoloid palpebral
slant exists (Figure 16). A straight edge held in front
of the palpebral fissure connecting the canthi may be
used to compare the relative canthal height. The nor-
mal relative canthal height depends on race. In
whites, the palpebral fissure is usually slightly mon-
goloid; that is, the lateral canthus is slightly higher
than the medial canthus. Careful measurements of the
Oriental palpebral fissure indicate less mongoloid
slant than would be expected from casual observa-
tion. The mongoloid slant illusion in many cases is
created by the absence of a skin fold in the upper lid
and from a superior epicanthal fold. Figure 15
The palpebral fissure configuration imparts a A esotropia in a patient with mongoloid palpebral fissures.
characteristic appearance to an individual including,
at times, a pseudostrabismus. This assumes clinical
significance because it is a common cause for referral
for suspected esotropia especially in infants.
Vertically incomitant strabismus (A and V patterns)
in esotropia seems to follow a pattern related to the
slant of the fissures. This pattern was first pointed out
by Urrets-Zavalia who noted that in esotropia a mon-
goloid fissure tends to be associated with an A pattern
and an antimongoloid fissure with a V pattern. He
found no similar correlation in exodeviations. When
examining a strabismus patient who has either a mon-
goloid or an antimongoloid lid fissure, vertical
incomitance should be looked for.
Patients with myelomeningocele demonstrate a
consistent abnormality in the configuration of the
palpebral fissure (Figure 17). What appears to be a
mongoloid slant in reality may be a straightening of
the lower lid margin, which gives the illusion of a
mongoloid slant. These patients frequently demon-
strate an A pattern usually associated with overaction
of the superior oblique muscle, and sometimes with
dissociated vertical deviation. The diagnosis of
myelomeningocele can be suspected in most cases
simply by looking at the lid configuration.

Figure 16
V esotropia in a patient with antimongoloid palpebral
fissures.

21
Chapter 2

Figure 17
A patient with myelomeningocele and a straight lower lid
margin simulating a mongoloid slant. This is a common
but unexplained finding in such patients.

Epicanthal folds
Epicanthal folds are present to some degree in
most infants and children during the first few years of
life (Figure 18 A, B). These skin folds can create an
illusion of esotropia. Parents think one eye turns in
B
because no white can be seen medially, especially in
the adducted eye in lateral versions. Two techniques
can be used to relieve parental concern regarding
pseudoesotropia from epicanthus. First, the examin-
er demonstrates the centered pupillary reflexes with a
muscle light. Second, the examiner carefully pulls the
skin forward over the bridge of the nose to demon-
strate the straightening effect of exposing the medi-
al conjunctiva or white of the eye (Figure 19). It is Figure 18
still a good rule for the ophthalmologist presented A Epicanthal folds obscure the nasal conjunctiva in both
patients, giving the appearance of esotropia. However,
with an obvious case of pseudostrabismus to carry out
the light reflex is centered in the pupil in each case.
a complete eye examination, including cycloplegic This reflex indicates the presence of parallel pupillary
refraction and retinal examination. A medial skin fold axes and, therefore, straight eyes or absence of
sweeping upward from below is called epicanthus manifest strabismus. Cover testing must be performed
eventually to confirm the presence of parallel visual axes
inversus (Figure 20).
because a large angle kappa* could hide a small
Telecanthus, which is an increased interorbital manifest esodeviation.
distance, may be confused with epicanthus (Figure B Epicanthal folds are present, but the displaced pupillary
21). Normally, the intercanthal distance is about one- reflex in the right eye confirms the presence of a right
esotropia.
half the pupillary distance. Intercanthal separation in
excess of this suggests true telecanthus, but this diag- *Angle kappa is the angle formed by the pupillary axis and the visual
nosis must be confirmed by radiologic evaluation axis. A positive angle kappa is present when the visual axis is nasal
to the pupillary axis. This simulates exotropia and is common. A neg-
demonstrating a bony abnormality. Other midline ative angle kappa is present when the visual axis is temporal to the
facial abnormalities, especially clefting of a facial pupillary axis. This simulates esotropia and is much less common
than positive angle kappa.
structure in the presence of telecanthus, should raise
the suspicion of defects at the base of the skull includ-
ing encephalocele. These patients also may have optic
nerve anomalies ranging from hypoplasia to morning
glory disk or even may be missing a medial rectus
muscle.

22
Surgical anatomy

A A

B B

Figure 19 Figure 21
A Centered pupillary light reflex A This patient demonstrates telecanthus with an
B The straightening effect of exposing more white nasal- interorbital dimension clearly more than one-half the
ly. (This is shown in an older patient because it is diffi- interpupillary distance and also an exotropia.
cult to photograph the younger child where the test is B This patient with telecanthus also has prominent
more effective.) epicanthal folds.

Conjunctiva
The bulbar conjunctiva, fused to the underlying
anterior Tenon's capsule, loosely covers the anterior
part of the globe from the fornices above and below
and from the canthi medially and laterally. The bul-
bar conjunctiva and anterior Tenon's capsule have
multiple, fine imbedded arterioles and veins. These
are branches of the anterior ciliary circulation and of
the marginal arcades of the vessels of the lids. Their
Figure 20 number and configuration vary from person to per-
A skin fold originating below and sweeping upward is called son. This circulation furnishes a small but probably
epicanthus inversus. This deformity is frequently significant blood supply to the anterior segment. The
associated with blepharophimosis and ptosis. These three
deformities, which may be combined with telecanthus, fused conjunctiva and anterior Tenon's capsule attach
cause significant disfigurement and present a formidable firmly to the sclera at the limbus (Figure 22). The
therapeutic challenge. combined conjuctiva and underlying anterior Tenon's
capsule is thick and has substance in infancy and
childhood but becomes much thinner and more fri-
able in adulthood and old age.
The plica semilunaris is a fold in the conjunctiva
located far medially in the palpebral fissure and is
mostly below the midline. The caruncle, located just
medial to the plica, is about 3 mm in diameter, cov-
ered with squamous epithelium, and often contains
small hairs (Figure 23). The relationship of the plica
and caruncle to each other and to the palpebral fissure
is an important cosmetic factor in strabismus surgery.
When repairing the conjunctiva, care should be taken
not to alter the position of these structures. It is par-

23
Chapter 2

During extraocular muscle surgery, all incisions


should be limited to the bulbar conjunctiva; they
should not extend into the fornix or palpebral con-
junctiva. An incision carried too deeply into the
fornix causes unnecessary bleeding and serves no
purpose. Transconjunctival incision in the palpebral
opening over the insertion of the medial or lateral rec-
tus should be avoided, if possible, because it can lead
to unsightly scarring, which in extreme cases can
even restrict motility.
When prior surgery has left the conjunctiva red-
dened and unsightly or scarred so that it limits motil-
A ity, the conjunctiva can be recessed with or without
Figure 22 removal of conjunctival tissue. In these cases, the
The topographic landmarks of the conjunctiva important to sclera is left uncovered. Sclera is rapidly re-covered
the strabismus surgeon are the following: with epithelium when it is left exposed, remaining
A The fusion of the conjunctiva and anterior Tenons comfortable in the process. It is not necessary to use
capsule with the sclera at the limbus
a mucous membrane graft to cover exposed sclera.
As long as one of the opposing surfaces behind the
lids is covered with epithelium, a symblepharon will
not develop.
A B
Tenons capsule
Tenon's capsule is a structure with definite
body and substance in childhood which gradually
atrophies in old age but not to the same degree as con-
junctiva. Tenon's capsule has an anterior and posteri-
or part. Anterior Tenon's capsule is the vestigial cap-
C
sulopalpebral head of the rectus muscles. This covers
the anterior half to two-thirds of the rectus muscles in
their sheaths as well as the intermuscular membrane.
Anterior Tenon's capsule is fused with the undersur-
face of conjunctiva and attaches to sclera at the lim-
bus. The fused conjunctiva-anterior Tenon's capsule
is movable over underlying posterior Tenon's capsule
Figure 23 and episclera, the latter being the anterior extension
A The limbus of posterior Tenon's capsule. Episclera starts at the
B The plica semilunaris level of the insertion of the rectus muscles in a line
C The caruncle
around the globe, which is called the spiral of Tillaux.
Episclera joins conjunctiva and anterior Tenon's cap-
sule, fusing at the limbus.
ticularly important that the plica not be displaced lat- Posterior Tenon's capsule is made up of the
erally, making it more obvious as a reddened, fibrous sheath of the rectus muscles together with the
unsightly mass seen in the palpebral opening. intermuscular membrane. According to Lester Jones,
A fat pad is present in the inferior fornix extend- the tissues that make up posterior Tenon's capsule
ing to within 12 to 14 mm of the limbus (Figure 24). form at a later evolutionary stage than those forming
This fat pad is beneath conjunctiva and its posterior anterior Tenon's capsule. Fibrous attachments
condensations behind the orbital septum, and is out- between the inner surface of anterior Tenon's capsule
side both layers of Tenon's capsule in the extraconal and the outer muscle sheath (part of posterior Tenon's
space. A transconjunctival incision made medially capsule) fuse at a point 15 to 20 mm behind the inser-
and laterally in the inferior cul-de-sac should be pos- tion of the medial and lateral rectus muscles to form
terior to the attachment of posterior Tenons capsule a barrier to extraconal fat. A condensation of fibrous
or at least 8 mm from the limbus in order to expose tissue and smooth muscle between the outer surface
bare sclera. However, it should also be anterior to the of anterior Tenon's capsule and the orbital wall medi-
inferior fat pad, no more than approximately 12 mm ally and laterally is the location of the aforementioned
from the limbus. There is no comparable fat pad pulleys of the horizontal rectus muscles. If the hori-
superiorly. zontal rectus muscle is separated completely from

24
Surgical anatomy

A B

C D

Fat Pad

Figure 24
A The shallow lower fornix with a visible fat pad beneath D Saggital section of the orbit shows the relationships of the
conjunctiva vertical rectus muscles, fat compartments, oblique
B The deep upper fornix with no visible fat under con- muscles, orbital septa, and lids. It should be noted that on
junctiva the superior aspect of the globe, the extraconal fat lies
C The inferior fat pad shown frontally above the levator palpebrae and behind the orbital
septum. Surgery on the superior oblique muscle is not
associated with extraconal fat because of the barrier of the
levator palpebrae muscle. Pulling the lower lid down
allows the inferior fat pad to prolapse.

anterior Tenon's capsule, exposing extraconal fat, disruption of the pulleys of the medial and lateral rec-
there will be no or reduced pulley effect on the eye tus muscles.
muscle. This will result in up and down slip of the While extraocular muscle surgery is performed
muscle relative to the globe. It is not practical or even beneath anterior Tenon's capsule, it is done within the
logical in the usual strabismus surgery to free pulleys plane of posterior Tenon's capsule. The intermuscu-
outside anterior Tenon's capsule, but this could be lar membrane part of posterior Tenon's capsule must
done for special need. Eye muscle surgery is routine- be fenestrated in order to place a muscle hook behind
ly performed entirely inside anterior Tenon's capsule the insertion of a rectus muscle (Figure 26 A-D).
with no fat exposure (Figure 25 A-C). How much more dissection is done in the intermus-
Posterior Tenon's capsule, composed of the cular membrane beyond the minimum required to
muscle's capsule and the intermuscular membrane, gain access to the muscle is the decision of the sur-
unites the rectus muscles in a ring around the globe. geon. It is probably wise to do as little cutting of pos-
The extent to which the intermuscular membrane is terior Tenon's capsule as is compatible with the con-
cut during surgery influences how far the rectus mus- duct of the surgical procedure intended. Retinal
cles, particularly the medial and to some extent the detachment surgery, in contrast to extraocular muscle
lateral, will retract during surgery. Dissection of pos- surgery, is carried out beneath posterior Tenon's cap-
terior Tenon's capsule far posteriorly leads to expo- sule. This enables a view of the scleral surface far
sure of intraconal fat, so called because it resides posteriorly to a point near the posterior ciliary vessels
inside the muscle cone. Excessive dissection of ante- and the optic nerve.
rior Tenon's capsule exposes extraconal fat and risks

25
Chapter 2

10
A

6, 4

Figure 25 The Conjunctiva/Tenons Capsule Relationships


A Axial view of the orbit B C Coronal section of B at X
1 Wall of the orbit 1 The limbal fusion of the conjunctiva 1 Conjunctiva
2 Conjunctiva and anterior Tenons capsule 2 Anterior Tenons capsule
3 Anterior Tenons capsule 2 Potential space between anterior 3 Muscle sheath
4 Posterior Tenons capsule Tenons capsule and episclera 4 Extraocular muscle
5 The muscle 3 The muscle in its sheath (posterior 5 Intermuscular membrane
6 Intermuscular membrane (posterior Tenons capsule) inserting into the 6 Sclera substance
Tenons capsule) sclera 7 Sclera surface
7 Intraconal orbital fat 4 Postinsertional muscle footplates
8 Extraconal orbital fat 5 Episclera
9 Horizontal pulley 6 Conjunctiva
10 Episclera 7 Anterior Tenons capsule

26
Surgical anatomy

B C

Figure 26
A When the layer of fused conjunctiva-anterior Tenon's B Posterior Tenons capsule attaches to sclera at the
capsule is retracted, the muscle insertion in its sheath is muscles insertion and in the intermuscular space
exposed. Fibrous attachments are seen between the forming the spiral of Tillaux.
undersurface of anterior Tenons capsule and the outer C The muscle hook is placed in a hole created in
surface of the muscle. The fusion of the intermuscular intermuscular membrane adjacent to the muscle
membrane (posterior Tenon's capsule), as well as of the insertion and glides along bare sclera behind the rectus
muscle to the sclera, is apparent. This fusion of the muscle insertion and is exposed at the opposite muscle
intermuscular membrane to the sclera must be incised border with a snip incision.
before the bare sclera and subposterior Tenon's capsule continued.
space can be encountered. Only after entering
subposterior Tenon's capsule space can the insertion of
the rectus muscle be engaged cleanly on a muscle hook.
This is the free space used by the retina surgeon. The
tip of the scissors in the photo points to this free space.

27
Chapter 2

Figure 26, contd


D The muscle hook is placed in a hole created in intermuscular membrane
adjacent to the muscle insertion and glides along bare sclera behind the
rectus muscle insertion and is exposed at the opposite muscle border with a
snip incision.With a limbal incision, the multiple layers and surfaces
associated with the rectus muscles can be readily seen. Conjunctiva and
anterior Tenons capsule shown here separated are actually fused and
separated only with difficulty.

Surgical anatomy of the rectus


muscles
Each rectus muscle inserts at a different distance muscle retroplacement from the muscle's actual inser-
from the limbus. The insertions of these muscles are tion can be noted by those surgeons accustomed to the
the prime surgical landmarks in extraocular muscle traditional medial rectus recession numbers used
surgery. The medial rectus is said to insert in the nor- as guidelines for recession. For example, if a 5.5 mm
mal eye 5.5 mm from the limbus. This figure pre- recession of the medial rectus is done in a patient
sumably was arrived at from study of otherwise nor- whose medial rectus inserts 4.5 mm from the limbus
mal eyes. Since no specific mention is made of (not noticed by the surgeon) and a 5.5 mm recession
whether the measurements were taken from speci- is done, the new insertion site is located 9.0 mm from
mens with strabismus, it is assumed they were not. the limbus in a normal sized eye. This could result in
The average distance between the limbus and the an undercorrection, and in all likelihood, this
medial rectus insertion of 112 medial rectus muscles occurred not infrequently when 5.5 mm was consid-
in 66 esotropic patients was 4.4 mm with a range of ered the maximum medial rectus recession. On the
3.0 to 6.0 mm. Eight patients had unequal medial rec- other hand, if in this same patient the medial rectus
tus insertion to limbus measurements. There was no were recessed 10.0 mm from the limbus, the resultant
correlation found between the angle of esodeviation recession measured from the insertion would actually
and the distance of the medial rectus insertion from be 6.5 mm, a number perhaps considered too large for
the limbus. The variability of this insertion along the deviation but one which would be required
with its lack of correlation with the angle of esotropia because of the medial rectus insertion site being clos-
begs the question, Is the insertion the best landmark er to the limbus.
for measurement of a medial rectus recession? Use of the limbus as the point of reference for
Since the answer is obviously no, it is preferable to medial rectus recession allows the surgeon to perform
use the limbus, a more consistent anatomical point, as larger recessions safely by not exceeding the land-
the reference for recession of the medial rectus mus- mark of the equator. The equatorial landmark has
cle. When measuring from the limbus, the amount of been shown to be reliable because in patients with

28
Surgical anatomy

refractive errors between + or - 4.00 diopters, the


axial length of the eye is predictable for the age of the
patient. This has been confirmed by simple to per-
form axial length measurement with the A-scan
device. At the same time, the corneal dimension is
also reliable. If it appears to be other than the normal
dimension, this is obvious and measurement for con-
firmation is simple. Whether or not discovery of the
pulleys will alter this thinking is not clear now. It is
known, however, that successful realignment of con-
genital esotropia occurs more frequently when meas-
urement is carried out from the limbus compared to
the prior upper limit of recession of 5.5 mm. With
larger medial rectus recession measured from the
insertion now being done, first surgery alignment in
congenital esotropia is improved, but the incidence of
overcorrection is not known.
The inferior rectus inserts 6.5 mm from the lim-
bus; the lateral rectus inserts 6.9 mm from the limbus
(range: 4.5 to 8.0 mm);* and the superior rectus Figure 27
The spiral of Tillaux and the relationship of the rectus
inserts 7.7 mm from the limbus. Beginning with the muscle insertions.
medial rectus and moving inferiorly and temporally,
each rectus muscle inserts farther from the limbus.
The line connecting these insertions is called the spi-
ral of Tillaux (Figure 27). The circumference of the
ring formed by closing the spiral is approximately 80
mm. The width of the insertion of each of the rectus
muscles is approximately 10 mm. The distance
between the adjacent insertion borders is approxi-
mately 10 mm (Figure 28).

The issue can be summed up as follows: the inser-


tion of the medial rectus muscle in esotropia tends
to be closer to the limbus than the 5.5 mm stated for
the normal. Therefore, recession measured from
the limbus, a more reliable landmark, allows larger
recessions to be done safely thus reducing the like-
lihood of undercorrection.

The insertion of the rectus muscles can be seen Figure 28


relatively easily through the intact conjunctiva. This Width of the rectus muscle insertions
means that the muscles location can be confirmed
when the eye is rotated and the conjunctiva is brought
tightly over the insertion of any of the rectus muscles.
Close observation reveals the line of insertion of the tion of the muscle to be operated upon. This maneu-
muscle, with the muscle appearing as a slightly dark- ver to establish the location of the rectus muscles
er and faintly raised structure beneath conjunctiva should be done routinely at the outset of each eye
(Figure 29). By confirming the rectus muscles inser- muscle surgical procedure.
tion in this manner, the surgeon can locate each of the The rectus muscles are all approximately 40 mm
rectus muscles accurately in roughly the 3, 6, 9, and long and each receives innervation from the under-
12 o'clock positions of the globe. This maneuver surface (intraconal space) at the junction of the mid-
leads to proper traction suture or traction forceps dle and posterior thirds of the muscle or 26 mm from
placement and allows strategic placement of the inci- the insertion. The six pairs of extraocular muscles are
sion through conjunctiva leading to accurate localiza- characterized in Table 1.

* Although the lateral rectus insertion site is variable, it is not common to measure from the limbus for recession of this muscle.

29
Chapter 2

Head Head

A B

Head Head

C D

Head

Figure 29
A The superior rectus muscle seen through the intact D The insertion of the medial rectus muscle seen through
conjunctiva and anterior Tenons capsule. the intact conjunctiva.
B The insertion of the inferior rectus muscle seen through E The insertion of the lateral and inferior rectus muscles
the intact conjunctiva. Note fat pad. seen through the intact conjunctiva with the inferior
C The insertion of the lateral rectus muscle seen through temporal orbital fat pad seen just inside the lower lid
the intact conjunctiva. margin. The site of the incision for inferior oblique
exposure is shown. This view is shown from above.

30
Surgical anatomy

Muscle Length(mm) Nerve Point of Innervation Tendon* (mm) Muscle action

Medial 40 III Inferior division 26 mm from insertion L: 3.7 Adduction


rectus (MR) W: 10.3

Inferior 40 III Inferior division 26 mm from insertion L: 5.5 Depression


rectus (IR) W: 9.8 Excycloduction
Adduction

Lateral 40 VI 26 mm from insertion L: 8.8 Abduction


rectus (LR) W: 9.2

Superior 40 III Superior division 26 mm from insertion L: 5.8 Elevation


rectus (SR) W: 10.8 Incycloduction
Adduction

Inferior 36 III Inferior division 12 mm posterior to insertion L: <1 Elevation


oblique (IO) of inferior rectus at its later- W: 9.4 Excycloduction
al border Abduction

Superior 60 IV 26 mm from trochlea L: 30 Depression


oblique (SO) W: 10.7 Incycloduction
Abduction

* L - length; W - width at insertion

Table 1 Extraocular Muscles

Characteristics of the Motor physiology


extraocular muscles Ficks axes - Listings lane
The extraocular muscles are similar to skeletal The theoretical center of rotation of the eye is at
muscles though there are differences undoubtedly the intersection of the three principal axes of Fick
related to the very specialized function of the extraoc- (Figure 30). The X-axis crosses the eye horizontally;
ular muscles. Both skeletal and extraocular muscles the Y-axis passes through the center of the pupil
have several types of twitch fibers, but the extraocu- (through the y sutures of the lens); and the Z-axis
lar muscles are unique, having tonically contracting crosses the eye vertically. Vertical movements take
fibers not found in skeletal muscle. The twitch fibers place around the X-axis, torsion takes place around
of extraocular muscles are called Fibrillenstruktur, the Y-axis, and medial and lateral rotation takes place
and the unique slow tonic fibers are called around the Z- axis. This is a simplistic but useful way
Felderstruktur. There are two muscle fiber layers in to characterize movements of the eye. However, the
the medial and lateral recti. The shorter orbital layer eye actually moves in Listings plane, responding to
inserts in the muscle pulley, and the longer global the summation of the actions of all of the extraocular
fibers insert into sclera at the muscles insertion. The muscles. All of the movements of the globe can be
muscle fibers are long, traversing the entire length of described as taking place in Listings plane. The new
the muscle, or in some cases, nearly so. The blood
supply of the extraocular muscles is rich, coming
from the muscular branches of the ophthalmic artery.
The extraocular muscles have the lowest innervation
ratio of any of the muscles of the body; that is, they
have the most nerve fibers per muscle fiber. This is
appropriate to meet the stringent requirements of
accuracy of fixation and smoothness of following
required to support a visual apparatus capable of both
rapid, accurate movement and sustained fixation.
There is evidence, not always corroborated but accu-
mulating nonetheless, showing that the extraocular
muscles participate in proprioception. Muscle spin-
dles and other muscle sensors communicate by means
of an inflow mechanism which is functional but
apparently less powerful than the outflow mechanism
Figure 30
generated from stimuli arising in the retina.

31
Chapter 2

description of the pulleys provides a useful concept globe, the insertion. The pulling effect on the globe
for appreciating Listings plane which appears to comes from the location of the pulley regardless of
coincide with the functional insertion of the rectus where the pull is initiated before it reaches the pulley.
muscles at the pulleys (Figure 31). This new post pulley concept replaces the earlier
held notion that the functional insertion of the
extraocular muscle was at the point of tangency with
the globe, which is anterior to the equator medially
A B and just behind the equator laterally. The new pulley
concept seems to be more compatible with the results
of strabismus management particularly dealing with
A and V patterns in comparison with those previ-
ously held (Figure 32).

Muscle forces
The extraocular muscles have a resting tension of
12 to 15 g. This tension increases to 40 to 50 g in the
agonist during a saccade. Tension in the antagonist
C
also increases somewhat during a saccade because of
the length tension effect and despite decreased inner-
vation. Maximum isometric contraction of an
extraocular muscle is approximately 125 g. The ten-
sion of the extraocular muscles when the eyes are
Figure 31 shifted away from the primary position and then sta-
A Saggital representation demonstrates how Listings bilized is greater than the tension in the primary posi-
plane coincides logically with the pulleys as the tion. When the eye is stabilized in any position; that
functional origin of the rectus muscles. is, not moving, tension is equal in opposing muscles.
B Frontal view of Listings plane
C Looking up to the left, the eyes move in Listings plane.
When the eyes are in a position away from the pri-
mary, static tension must be increased compared to
the primary or neutral position. This is to overcome
the passive forces of the orbital fascia which must be
Pulleys deflected to maintain position of the eye away from
The extraocular muscles initiate ocular move- the primary position. When the eye muscles are in
ment and then sustain a new position of the globe the resting state under general anesthesia, the eyes are
through a complex transfer of energy. The insertion more or less centered in the palpebral fissure or they
of each extraocular muscle on the globe acts on what may be slightly exotropic. If the eyes are passively
we tend to think of as a point-to-point basis. The moved from this position, they will spring back. This
actual muscle-globe relationship, however, is mediat- action is the basis for the spring back balance test of
ed through a complex arrangement of fascial attach- Jampolsky. This is a technique for evaluating the bal-
ments, including anterior and posterior Tenon's cap- ance of purely passive forces in ocular alignment and,
sule, conjunctiva, and numerous attachments between therefore, a guide to surgery in cases with mechanical
these structures. Initiation of the globe's movement restrictions.
can only be carried out by the action of the muscles. During pursuit movements, both eyes move at
This movement can be slowed or stopped by the pas- the same, usually moderate, speed, and in the same
sive fascial structures, including fat that surround the direction: right, left, up, down. While carrying out
globe and extraocular muscles. Movement of the vergence movements, eye movement is slower and in
globe stopped by passive fascial structures is useful opposite directions. During convergence (e.g. the
physiologically, or harmful in pathologic states. right eye moves to the left and the left eye to the
Normal ocular movements are stopped by mechanical right), both eyes are looking toward the nose, so to
factors in the extremes of abduction, adduction, ele- speak. The opposite takes place during divergence,
vation, and depression. These mechanical factors are although pure divergence amplitude is limited and
the check ligaments associated with the pulleys or occurs in the normal only when stimulated. When the
pulley-like structures that are formed by fascial con- eyes suddenly change fixation from one object to
densations between anterior Tenon's capsule and the another, a rapid movement or saccade takes place.
periorbita. In simple terms, the muscles act like a The two eyes normally move at the same speed and in
rope passing through a pulley on their way to attach- the same direction at speeds up to 250 to 400
ing to the globe. The pull of the muscle on the globe degrees/sec. This saccadic speed is reduced in a
occurs at the point where the muscle attaches to the paretic muscle more or less proportional to the degree

32
Surgical anatomy

Figure 32
A The medial rectus inserts between 3 and 6 mm from the C In the pathologic state, restriction in any of the inner
limbus. fascial structures around the globe may limit passive
B Pulley tissues also act as check ligaments at extremes ductions. In the presence of a normal agonist, these
of gaze. abnormalities usually result in increased intraocular
pressure and changes in the palpebral fissure.

33
Chapter 2

of muscle weakness. Reduction of speed of a saccade Underaction and overaction


is a fairly accurate estimate of the extent of weakness
of a muscle. The slower a saccade, compared to the When eye movements, versions or ductions are
normal fellow eye, the weaker the muscle in question. evaluated clinically, reference is made to overaction
The human extraocular muscle is richly innervated. or underaction of a muscle. Underaction of the
The ratio of nerve fibers to muscle fibers in the extraocular muscles can be readily explained due to
extraocular muscle is 1:5 compared to 1:120 in skele- reduced rotation of the globe. This is from decreased
tal muscle in the leg. innervation, loss of muscle substance, or because of
an altered position of the muscle on the globe.
Apex of the orbit Underaction can also result from tightness or tether-
The eyes move in the manner of a servomech- ing of the passive fascial structures and have no rela-
anism, that is, a given event predictably produces an tion to how effectively the muscle can pull. When a
appropriate response. For example, shift of the object normal muscle contracts in the presence of a restric-
of regard to the temporal retina of the right eye and tion, a rise in intraocular pressure will result. For
nasal retina of the left eye produces just enough lev- example, in cases of thyroid myopathy involving the
oversion to place the object of regard on the foveas. inferior recti, patients have been treated for glauco-
Bitemporal retinal disparity will cause convergence, ma because of the elevated intraocular pressure which
for example. occurred during attempts to look up. Underaction
The extraocular muscles have their anatomic ori- associated with paresis or paralysis of the agonist can
gin around the ligament of Zinn at the apex of the be shown by observing a saccade. In this case, the
orbit (Figure 33). This site is also the point of entry saccadic velocity will be reduced and no increase in
of the major nerves and blood vessels supplying the intraocular pressure will be noted when movement is
orbit and globe. Knowledge of these structures and attempted in the restricted field. Underaction of the
their relationships is vital to understanding many superior oblique can result from a congenitally elon-
pathologic conditions in and around the orbit. gated tendon with a normal saccade. This will be dis-
cussed in detail later.

Figure 33
The configuration of structures passing through the optic foramen, superior and inferior orbital fissure, and annular ligament
of Zinn of the left eye.

34
Surgical anatomy

Overaction of an extraocular muscle is more The inferior oblique muscle was subjected to sur-
accurately described in most cases not as over exu- gery as early as 1841, but for myopia! By 1885, infe-
berance of the muscle but as underaction of the pas- rior oblique weakening for treatment of superior
sive checking tissue. Observation of clinical strabis- oblique palsy was done. The muscle was exposed
mus suggests that only cases of excess innervation through a skin incision and the muscle was cut medi-
such as occurs in the yoke muscle of a paretic muscle al to Lockwood's ligament. This technique persisted
can legitimately be called overaction. In spite of the into the middle of the twentieth century. After that,
obvious misnomer, the term overaction is firmly more attention was directed to weakening the muscle
implanted in the literature and the language of the distal to Lockwood's ligament nearer the insertion.
strabismologist. Currently the most effective techniques for inferior
oblique weakening include myotomy, myectomy, and
Surgical anatomy of the recession, placing the new insertion at various posi-
inferior oblique tions in the inferior temporal quadrant according to
The inferior oblique muscle is 36 mm long. It the surgeon's preference. Surgical techniques for
originates a few millimeters behind the medial end of inferior oblique weakening also vary in the extent to
the inferior orbital rim just lateral to the lacrimal which the inferior oblique is freed from its union with
fossa and proceeds posteriorly and temporally at an Lockwood's ligament and in the management of the
angle of 51 degrees with the frontal plane passing neuro-vascular bundle.
beneath the inferior rectus (between the inferior rec- In order to avoid surgery on the inferior oblique
tus and the floor of the orbit) (Figure 34). It inserts in some cases of overaction, Bielschowsky was said
beneath the inferior border of the lateral rectus mus- to have lowered the medial rectus. This portended
cle, approximately 12 mm from the insertion of the treatment of A and V pattern with vertical shift of
lateral rectus. The posterior extent of the inferior the rectus muscles and possibly the description of the
oblique insertion overlies a point 2 mm below and 2 pulleys of the rectus muscles whose anomalous loca-
mm lateral to the macula. The middle of the distal tion leads to vertical incomitance.
half of the muscle covers the inferior temporal vortex Recently, inferior oblique anterior transposition
vein. The blood vessels in the inferior oblique do not has been used for the treatment of inferior oblique
contribute to the blood supply of the anterior segment overaction especially when it is associated with dis-
of the globe. This muscle receives its innervation on sociated vertical deviation (DVD). The mechanism
its upper surface at the point where it passes beneath of action for this treatment of DVD has been
the lateral border of the inferior rectus, approximate- explained by Stager who demonstrated that the robust
ly 12 mm posterior to the lateral corner of the inser- neurovascular bundle of the inferior oblique is effec-
tion of the inferior rectus. The inferior oblique mus- tive in anchoring the transposed inferior oblique
cle is unique in its anatomic relationships. This mus- insertion. This is the same neurovascular bundle that
cle behaves as though it has two potential insertions was cut by Parks during the denervation and extirpa-
and two potential points of origin. Because the infe- tion procedure for maximum weakening of the inferi-
rior oblique is innervated near its middle, it may be or oblique.
weakened either proximal or distal to its point of Myectomy or large recession of the inferior
innervation. oblique distal to the muscles attachment at

A B

Figure 34
The inferior oblique (A) from in front and (B) from behind.

35
Chapter 2

Lockwood's ligament makes this attachment equiva- oblique. The descriptive term elevation in adduc-
lent to the new functional insertion (Figure 35). tion describes a condition where the inferior oblique
Although not done now, earlier procedures for weak- is responsible for elevation, not necessarily from its
ening the inferior oblique, which were carried out overacting but rather from the lack of checking from
nasal to the ligament of Lockwood, meant that the a weak (or absent) superior oblique. In defense of the
inferior oblique union with Lockwoods ligament term overaction of the inferior oblique, this term
became the functional origin. A procedure described also describes the extorsion and abduction caused by
by Stager and Weakley transected the inferior rectus the inferior oblique in cases of anomalous orbital
on both sides of Lockwood's ligament relying on a anatomy and/or upshift of the medial rectus pulleys
small segment of the middle of the muscle stabilized and in cases of deficient adduction.
by Lockwood's and the neurovascular bundle. In
cases of extirpation and denervation of the inferior Lockwoods ligament
oblique, a large myectomy of the distal inferior Lockwoods ligament may be compared to a
oblique is combined with transection of the neurovas- hammock supporting the globe (Figure 36). It forms
cular bundle. a dense condensation of tissue that engulfs the inferi-
The inferior oblique is unique among the or rectus and inferior oblique muscles beneath the
extraocular muscles in that, in many cases, weaken- globe. The attachment of Lockwoods ligament to the
ing of this muscle, even by extensive surgery, seems inferior oblique affects globe movement from the
to have relatively little effect on movement of the inferior oblique muscle when it contracts, even when
globe or alignment of the eyes. Even after large the inferior oblique is transected on both sides of
recession or myectomy, apparent overaction of the Lockwoods!
inferior oblique can persist. This is probably due to Attachments between Lockwoods ligament
horizontal rectus action from upward pulley displace- and neighboring muscle and fascial structures are
ment of the medial rectus. Also, in the relatively connected to the lower lid. This makes lower lid pto-
uncommon inferior oblique paresis, strabismus is sis a potential complication of inferior rectus reces-
much less than would occur after paresis of any of the sion (Figure 37). To avoid this, the inferior rectus
other muscles. Effective weakening of this muscle should be freed extensively during surgery. Guyton,
could be made more difficult because of the unique et. al., have recommended that Lockwoods ligament
anatomy. Likewise neurologically, the muscles be advanced when recession of the inferior rectus
innervation by the inferior branch of cranial nerve III muscle is carried out. When resection of the inferior
makes isolated paralysis rare. In contrast, the inferi- rectus is performed, persistent attachment of this
or oblique seems to overact commonly. But is muscle to Lockwoods ligament can cause just the
overaction the right term? Some think it is not, sug- opposite, a bothersome and cosmetically unaccept-
gesting that the preferred term would simply describe able elevation of the lower lid resulting in narrowing
appearance not etiology. The term "elevation in of the palpebral fissure. Freeing the inferior rectus
adduction," which replaces the Latin "strabismus sur- muscle from Lockwoods ligament also helps avoid
soadductorius," seems to be a valid description of this complication.
what has been called overaction of the inferior

Figure 35
The inferior oblique behaves as if it had two potential origins and two potential insertions because of its union with
Lockwood's ligament as it passes beneath the inferior rectus. In addition, at the mid-section of the inferior oblique is a stout
neurovascular bundle, described in detail by Stager and associates, which acts both as a restraining anchor and a source
of innervation.

36
Surgical anatomy

A B

Figure 36
A The ligament of Lockwood could be compared to a C The inferior fat pad is prominent and should not be
hammock supporting the globe. disturbed during surgery of the inferior rectus.
B The inferior oblique passes beneath the inferior rectus,
through Lockwoods ligament and orbital fat
approximately 12 - 14 mm from the limbus.

Figure 37
A saggital section of the complex anatomy of the orbit shows the intimate relationship of the inferior rectus, inferior oblique,
and Lockwoods ligament. This complex, in turn, is connected to the lower lid tarsus and inferior orbital septum. The
inferior extraconal fat protrudes farther forward compared to the extraconal fat of the superior globe. Recession of the
inferior rectus causes recession of the lower lid and widening of the fissure. Advancement or resection of the inferior rectus
causes narrowing of the palpebral fissure. Placement of the conjunctival incision too far from the limbus inferiorly can result
in disturbance of the extraconal fat compartment.

37
Chapter 2

Superior oblique
The superior oblique muscle has a muscular por-
tion and a tendinous portion, both of which are
approximately 30 mm long. The muscle portion orig-
inates superiorly and nasal to the ligament of Zinn at
the apex of the orbit and becomes tendinous 10 mm
before reaching the trochlea. The trochlea, a carti-
laginous saddle-shaped structure, is located at the
junction of the medial and superior orbital rim just
posterior to the orbital rim. The trochlea acts as a pul-
ley redirecting the course of the superior oblique ten-
don. approximately 54 degrees from the frontal plane.
The tendon passes beneath the superior rectus, insert-
ing under the lateral border of the superior rectus usu-
ally 5-7 mm posterior to the temporal superior rectus
insertion or approximately 13 mm from the limbus
(Figure 38). That portion of the superior oblique ten-
don passing beneath the superior rectus muscle is
attached to the undersurface of this muscle through
the common sheath of the superior rectus muscle. Figure 38
The superior oblique tendon is redirected to 54 from the
Therefore, to obtain an effective large recession of the frontal plane and passes posteriorly and temporally beneath
superior rectus muscle, it is logical to free it from the the superior rectus.
superior oblique tendon. A hang loose superior rectus
recession which is not secured at the intended rein-
sertion site may not accomplish the intended retro-
placement of the superior rectus muscle unless the
superior oblique - superior rectus attachment is freed.
The diameter of the superior oblique tendon
between the trochlea and the medial border of the
superior rectus is about 3 mm. The tendon is white,
surrounded by dense fascia, and lacking a discreet
tendon sheath. Because of this fascia, the superior
oblique tendon nasal to the superior rectus can be
somewhat difficult to identify when approached out-
side of anterior Tenons capsule. However, when
approached from the undersurface of anterior Tenons
capsule, the superior oblique tendon is an easily dis-
tinguishable structure (Figure 39). The nerve to the
superior oblique enters the muscular portion 26 mm Figure 39
From Parks MM, Helveston EM. Direct visualization of the
posterior to the trochlea. Blood vessels in the superi-
superior oblique tendon. Archives of Ophthalmology, 1970,
or oblique do not contribute to the blood supply of the 84:491-494. Used with permission.
anterior segment of the globe .
The insertion of the superior oblique is broad,
measuring on average 10.7 mm. The fibers at this
point are very thin and fuse with sclera in a manner
that makes the superior oblique insertion difficult to
distinguish from sclera. Only after carefully looking
in the area where the superior oblique should insert
can these fibers be seen coursing temporally and
slightly posterior. At times the surgeon must employ
a fine hook to carefully tease the insertion into view.
The insertion of the superior oblique has been shown
to be the most variable of any of the extraocular mus-
cles. While the tendon usually inserts at the lateral
border of the superior rectus muscle about 5 to 7 mm Figure 40
behind the superior rectus insertion, the superior The 'normal insertion of the superior oblique varies. The
insertion displaced medial to the superior rectus results in
oblique tendon can be found more anterior. In other
underaction.

38
Surgical anatomy

cases, the superior oblique can be found inserting at the sclera, as in hang-loose recession, an unpre-
the medial border of the superior rectus. This results dictable result may occur simply on the basis of the
in superior oblique underaction with excyclotropia anatomy. The union of the superior oblique tendon
because this nasal displacement reduces the torsional and superior rectus could pull the superior rectus for-
effect of the superior oblique. A wide range of other ward toward the limbus as the eye rotates from
anomalies is seen with the superior oblique tendon depression to the primary position (Figure 42).
from laxity causing congenital superior oblique Therefore, the hang-loose recession of the superior
palsy to absence of the reflected tendon. This spec- rectus muscle used in cases of dissociated vertical
trum of anomalies suggests a new way of classifying deviation lacks a sound anatomic basis.
congenital superior oblique palsy (see page 157). In order to recess the superior rectus more than 5
The superior oblique tendon joins with the or 6 mm with a hang-loose, the superior rectus
undersurface of the superior rectus muscle by a com- should be freed from the superior oblique. For a
mon muscle-tendon capsular attachment. This attach- recession as great as 10 mm as some claim with the
ment can be seen clearly when the superior rectus hang-loose, the superior rectus insertion must be
muscle has been detached from the globe and lifted behind the path of the superior oblique. Prieto Diaz
upward (Figure 41). The firmness of this attachment demonstrated with x-ray a 15 mm recession of the
varies, but these two structures do not appear to be superior rectus from the limbus in down gaze after
entirely free of any connection to each other in the hang-loose of the superior rectus. Would this reces-
normal state. As stated above, if recession of the sion be as large with the eye in primary position?
superior rectus is attempted without securing the
superior rectus at the intended site of reattachment to

Figure 41
A The superior oblique passes beneath the superior rectus B The superior oblique remains attached to the superior
rectus when the rectus is detached and pulled up.

39
Chapter 2

Figure 42
A When the eye is rotated downward, the superior rectus B When the eye returns to the primary position, the
is the intended distance in a very large hang loose superior rectus could be pulled forward, reducing the
recession even if the superior oblique tendon - superior amount of recession.
rectus union is intact.

40
Surgical anatomy

Whitnalls ligament
Whitnalls (superior transverse) ligament and the usually results. Therefore, it is safer to hook the supe-
superior oblique tendon in the trochlea have common rior oblique tendon under direct vision. This can be
fascial attachments at the orbital rim (Figure 43). If done between the nasal border of the superior rectus
the superior transverse ligament is weakened inadver- and the trochlea or an even safer place is at the inser-
tently while hooking the superior oblique tendon, tion. Whitnalls ligament acts as a clothesline, sus-
thereby weakening the medial horn of the levator pending the levator aponeurosis and the medial por-
muscle, ptosis of the nasal portion of the upper lid tion of the superior oblique tendon.

A B

Figure 43
A The relationship of Whitnalls ligament and the superior C Nasal ptosis right eye from disruption of Whitnalls
oblique tendon. Blind hooking the superior oblique ligament after hooking of the superior oblique tendon in a
tendon can damage Whitnalls, producing ptosis. blind sweep nasal to the superior rectus.
B Whitnalls ligament acts like a clothesline with orbital
structures suspended.

41
Chapter 2

Trochlea
The trochlea remained the largest undescribed the trochlea is the functional origin of the superior
portion of human anatomy until 1982. I began the oblique muscle (actually the superior oblique ten-
definitive study of the human trochlea with the origi- don).
nal exenteration specimen shown below that con- The tendon of the superior oblique can tele-
tained the superior oblique tendon, trochlea, and the scope inward toward the apex of the orbit approxi-
distal superior oblique muscle, all in their physiolog- mately 16 mm during maximum downgaze in adduc-
ic relationships* (Figure 44). The intact nature of the tion and telescope 16 mm outward in maximum
specimen allowed separation of the structures com- upgaze in adduction. Tendon movement cannot
prising the superior oblique complex as shown. This exceed these limits because the peripheral superior
specimen was carefully dissected and extensively oblique tendon fibers are attached to the trochlea.
recorded including videotaped images showing the The multiple fiber layers making up the superior
way in which the superior oblique tendon passed oblique tendon slide with a cumulative effect with
through or more accurately slid in a telescoping man- only the central fibers carrying out the maximum
ner inside the trochlea. These studies confirmed that excursion (Figure 45).

Figure 44
A Exenterated orbit specimen containing the superior B The trochlear complex including: 1) distal muscle,
oblique complex 2) trochlea, and 3) proximal tendon. These were
dissected from exenteration specimen.

* Helveston EM, Merriam WW, Ellis FD, et. al. The trochlea: a study of the anatomy and physiology, Ophthalmology,
89(2):124, 1982.

42
Surgical anatomy

A B

Figure 45
A In downgaze the direction of movement of superior C In upgaze the direction of movement of superior oblique
oblique tendon fibers tendon fibers
B Location and distance of movement of the superior
oblique insertion in: 1) downgaze, 2) primary position,
and 3) upgaze

43
Chapter 2

Reasons why the trochlea remained unde- by study of autopsy specimens demonstrated that the
scribed and undisturbed during the course of strabis- bulk of the trochlea is made up of a cartilage saddle
mus surgery for so long include its location just inside 5.5 mm long, 4 mm thick, and 4 mm wide with a
the superior orbital rim and its close relationship to groove facing the orbital wall and with a curve con-
the superior orbital vessels and nerves (Figure 46). vexed toward the bone (Figure 47). Scanning elec-
The superior oblique tendon transfer procedure, as tron microscopy demonstrates the following trochlear
first described, did include subperiosteal dislocation components (Figure 48):
of the trochlea through a skin incision, but there is no 1) Cartilage saddle
evidence that the trochlea itself was seen when this 2) A bursa-like space on the bearing surface
procedure was done. Most anatomic drawings rep- between the tendon and the groove in the
resent the trochlea in a stylized fashion, portraying it cartilaginous saddle
as a sling through which the tendon passes freely or 3) A fibrillar-vascular structure surrounding
else it is shown as a lump with the tendon entering the superior oblique tendon
on one side and exiting on the other. 4) The superior oblique tendon
Study of the trochlea first made possible by the 5) Fibrous bands attaching the trochlea to the
exenteration specimen shown in Figure 44 and later bone of the orbit

A B

Figure 46
A The trochlea attached to the medial orbital wall with the B With fascial tissues removed the superior oblique tendon
tendon entering and exiting. seen exiting the trochlea through a cuff attached to the
trochlea.

A B

Figure 47 Dimensions of the trochlea


A Saggital B Coronal

44
Surgical anatomy

Figure 48
1) cartilage saddle, 2) bursa, 3) fibrillar - vascular space, 4) tendon, 5) attachment to orbital rim

The superior oblique tendon in the trochlea is Craig Gosling of the medical illustration department
made up of approximately 270 bundles of fibers. In at the Indiana University School of Medicine demon-
the several specimens studied, individual fibers in the strates the proposed dynamic relationship of the com-
bundles range in size from 0.01 to 0.1 mm. The fibers ponents of the trochlea. Significantly, the superior
appear discreet and flattened or triangular (Figure oblique tendon fibers appear to slide by each other
49). with a definite limit for each fiber, meaning that the
A description of the conclusions of the work more central fibers move farther than the more
describing the form and function of the trochlea is peripheral fibers and that the tendon moves with a
depicted in a composite drawn schematically by cumulative effect (Figure 50).

Figure 49
Scanning electron microscopy view of superior oblique tendon x320

45
Chapter 2

Figure 50 Composite

In the combined experience of strabismus sur- When these patients were studied retrospective-
geons, the superior oblique muscle has been found to ly, they had, in addition to their apparent superior
be anomalous more frequently than any other oblique palsy, a higher incidence of amblyopia and/or
extraocular muscle. The insertion varies widely in its horizontal strabismus compared to patients with
location. More importantly, absence of the superior superior oblique palsy in whom a superior oblique
oblique tendon in cases of superior oblique palsy tendon was found. Only 1 of 28 patients with congen-
has been observed and reported. In most of these ital superior oblique palsy who had a superior oblique
cases, a diagnosis of unilateral or bilateral superior tendon at surgery had amblyopia and/or horizontal
oblique palsy was made and surgery was undertaken strabismus. Also, all patients who eventually had
with the intention of doing a tuck of the superior absence of one or both superior oblique muscles had
oblique tendon. If a careful search at the insertion pronounced underaction of the superior oblique mus-
reveals no superior oblique tendon in these cases, the cle on the involved side preoperatively. Absence of
incision should be enlarged, the superior rectus the trochlea and superior oblique muscle has been
detached, and the sclera inspected from the superior demonstrated on CT (Figure 52). Patients with a
border of the horizontal recti to several millimeters diagnosis of congenital superior oblique palsy, with
posterior to the equator, including the entire anterior- or without a superior oblique tendon, have in com-
superior globe. If no superior oblique tendon is mon a superior oblique traction test suggesting a lax
found, an inferior oblique myectomy is carried out. or absent tendon and are likely to have facial asym-
Also performed is a recession of the yoke of the metry, with the larger face on the side of the paretic or
absent superior oblique, the contra lateral inferior rec- absent superior oblique.
tus, and/or recession of the ipsilateral superior rectus
(Figure 51).

46
Surgical anatomy

Figure 51
A Gaze positions showing overaction of the right inferior B At surgery, absence of the right superior oblique tendon
oblique and underaction of the right superior oblique. was confirmed.

Figure 52
A CT scan showing trochlea on the left and no trochlea on B Same patient demonstrating the superior oblique muscle
the right. on the right and no muscle on the left.

47
Chapter 2

Anterior segment blood supply


The principal blood supply to the anterior seg- coma. Patients achieved lowered intraocular pressure
ment of the eye is from the anterior ciliary arteries, without experiencing the typical clinical signs of
which travel in the four rectus muscles with anasto- anterior segment ischemia.
moses to the conjunctival and anterior Tenons ves- The medial anterior segment circulation is the
sels (Figure 53). These arteries are the major blood most protected because it is supplied by two anterior
supply to the anterior segment. The rest of the blood ciliary arteries and a long posterior ciliary artery
to the anterior segment is supplied by the two long (Figure 55). The superior and inferior quadrants are
posterior ciliary arteries that come forward intrascle- the least protected because they have no long posteri-
rally at the 3- and 9-oclock positions. Both sets of or ciliary artery. In clinical practice, it is not known
arteries originate from the ophthalmic artery. Each of with certainty what factors ultimately influence the
the rectus muscles has two anterior ciliary arteries, postsurgical dynamics of anterior segment circulation
with the exception of the lateral rectus which has one. in a given case. Some useful guidelines follow:
These arteries lie within the muscle until near the ten- 1. When a muscle is detached and reattached,
don when they exit the muscle and travel in the mus- anterior ciliary vessels do not recannulate.
cle capsule. There they are visible until reaching the 2. Because there are no long posterior ciliary
muscles insertion where they enter sclera to form the arteries superiorly or inferiorly, detachment
episcleral circle. From the episcleral circle, blood of the superior or inferior rectus muscles
flows to the intramuscular circle and from there flows disrupts iris vessel filling more than detach-
into multiple branches of the major arterial circle sup- ment of the horizontal recti.
plying blood to the ciliary muscle, ciliary process, 3. Older, vascularly-compromised patients may
and iris. This circle is discontinuous. The anterior be more likely than young, healthy patients
ciliary arteries furnish 70 to 80% of the blood supply to develop anterior segment changes after
to the anterior segment. The long posterior ciliary eye muscle surgery.
artery bypasses the episcleral circle and joins the 4. Iris angiography is a valid way to assess
intramuscular circle. The recurrent ciliary artery anterior segment circulation at a given time,
serving the choroid is supplied by the intramuscular but it is not a valid or practical predictor to
circle. The long posterior ciliary arteries provide less determine what might happen if eye muscles
than 30% of the blood supply to the anterior segment. are detached.
Of the two long posterior ciliary arteries, the medial 5. Anterior segment ischemia is rare, fortu-
provides the most blood (Figure 54). nately.
As a rule, at least one anterior ciliary artery 6. If anterior segment ischemia occurs, it
should remain undisturbed by strabismus surgery to should be treated with atropine and frequent
avoid anterior segment ischemia. However, anterior instillation of topical steroids.
segment ischemic changes have been seen after as
few as two muscles have been detached. Conversely,
all four muscles have been detached at one procedure
or in two, staged procedures separated by months or
years without producing anterior segment ischemia,
in some cases. Orge, et. al. have shown that detach-
ment of rectus muscles can reduce blood flow in the
ophthalmic artery 30%, presumably because of
downstream effects. Such a change in susceptible
individuals could lead to acute anterior segment
ischemia.
There is no evidence that severed anterior ciliary
arteries ever recanalize to nourish the anterior seg-
ment, but time could be a factor in establishing col-
lateral circulation. The conjunctival circulation and
its contribution to the anterior segment have been dis-
cussed as factors that influence the type of conjuncti-
val incision to be made. The cul-de-sac incision,
which disrupts conjunctival circulation less than the
limbal incision, has been suggested as being safer. In Figure 53
contrast, Awaya has detached all four rectus muscles There are seven anterior ciliary arteries, two each in the
at a single procedure to lower intraocular pressure as superior, inferior, and medial rectus muscles. The lateral
rectus has one. The anatomy of these vessels is subject to
an alternative to cyclocryotherapy in advanced glau- marked variation.

48
Surgical anatomy

Figure 54
Schematic of the blood supply of the anterior segment from Saunders, et. al.
ACA = anterior ciliary artery IMC = intramuscular circle
LPCA = long posterior ciliary artery RCA = recurrent choroidal artery

From Saunders RA, et al. Anterior segment ischemia after strabismus surgery. Survey of Ophthalmology, 1994, 38(5):456-
466. Used with permission.

A B

Figure 55
A Normal iris filling after preoperative intravenous injection B First postoperative day after detachment and transfer of
of fluorescein in a 30-year-old man. the superior and inferior rectus muscles. Note superior
and inferior sector filling delay.
continued.

49
Chapter 2

Figure 55, contd


From Olver J, Lee JP: Eye 3:318-326, 1989.

A variety of eye muscle transfer procedures,


including the Jensen tendon-muscle splitting transfer,
are designed to spare one anterior ciliary vessel in
each of the split muscles. However, von Noorden has
pointed out that the surgeon should look for anom-
alous anterior ciliary vessels that may crowd to the
half of the muscle that is pulled over for the transfer
and should then avoid ligating both vessels since this
would leave no anterior ciliary vessel in the undis-
turbed portion of the muscle.
Both McKeown, et. al., and Roth, in separate
papers published in 1989, described a technique for
detaching a rectus muscle while sparing the anterior
ciliary arteries (Figure 56). The technique requires
meticulous dissection aided by magnification with the
operation microscope. Because of the low incidence Figure 56
of clinically significant anterior segment ischemia The anterior ciliary arteries are dissected from the
and the difficulty of this procedure, its use is likely to superficial capsular muscle tissue allowing repositioning of
be highly selective. the muscle while leaving blood flow undisturbed.

50
Surgical anatomy

Vortex veins
There are normally four vortex veins in each eye. are observed in almost every case of inferior rectus
They are located roughly equidistant in the quadrants surgery. This occurs because the inferior vortex veins
of the globe; that is, 90 degrees apart (Figure 57). are situated about 1 mm closer to the midline near the
These veins drain blood from the iris, ciliary body, equator. Dissection of the inferior rectus is usually
and choroid. Their appearance is variable and rarely carried posteriorly to a point often posterior to the
will they number greater than four. These veins have inferior vortex veins to limit the lid effects of
a tortuous 5-7 mm intrascleral course and a similar Lockwoods ligament. The inferior temporal vortex
extrascleral course before passing through posterior vein is encountered in nearly every case when engag-
Tenons into the intraconal space. The superior vor- ing the inferior oblique in the inferior temporal quad-
tex veins empty into the superior orbital vein, and the rant. It is rare to encounter a vortex vein during sur-
remaining vortex veins empty into the inferior orbital gery on the superior or lateral rectus.
vein. If the surgeon exercises reasonable care, the vor-
In the course of strabismus surgery, each of the tex veins will remain intact. They look vulnerable but
vortex veins seems to have its own personality are actually fairly resistant to careful manipulation.
(Figure 58). The superior temporal vortex vein is Rupture of a vortex vein is rare, if surgery is done
seen at the posterior insertion of the superior oblique carefully. If a vortex vein is ruptured, it is treated
tendon. This is a reliable finding. The superior nasal with compression, and if necessary, cautery. A great
vortex vein and/or the inferior nasal vortex vein may deal of discoloration and swelling will occur, but
be seen while recessing the medial rectus, but rarely. there is no lasting complications to the surgery.
The inferior nasal and inferior temporal vortex veins

A B

Figure 57 The four vortex veins are viewed from the posterior aspect of the globe.
A Lateral B Medial

A B

Figure 58
A The superior temporal vortex vein is seen at the posterior B A vortex vein may be seen but rarely at either (or both)
insertion of the superior oblique. Vortex veins are not borders of the medial rectus.
seen routinely during surgery on the superior rectus. continued.

51
Chapter 2

C D

Figure 58, contd


C A vortex vein is seen routinely under the mid-belly of the D Vortex veins are seen at one or both borders of the
distal inferior oblique. inferior rectus.

Orbit and extraocular muscle


imaging
Two widely used techniques for imaging the
orbit and the extraocular muscles are computed A
tomography (CT) and magnetic resonance imaging
(MRI) (Figure 59). CT utilizes high resolution x-rays
recorded in fine cuts. CT is superior to MRI for
detecting calcium within a lesion or bony changes
such as with an orbital fracture and in identifying
early invasion or modeling from an adjacent lesion.
Iodine-containing contrast material can aid the diag-
nostic capability of CT but possible side effects could
be serious.
MRI provides high soft tissue contrast along with
high spatial resolution achievable in multiple planes
giving excellent anatomic detail of soft tissues. The
MRI utilizes a strong magnetic field to align a portion
of the nuclear spin that is ordinarily random. Using
the aligned proton as a target, the field is exposed to
pulsed energy that momentarily allows the proton to
B
resume its usual position only to become realigned at
the end of the pulse. The energy given off during
realignment enables the signal which in turn produces
the image. The pulse sequences are called T1 and T2.
Fluid is dark on T1 weighted images, and fat is bright.
Fat is darker and less distinct on T2 weighted images
(Figure 60). Various techniques are available to sup-
press the fat with T1 images thus providing a better
image of some orbital contents.
For viewing the extraocular muscles, T1 weight-
ed images of dark muscles are satisfactory. The
views may be coronal, axial, or saggital (Figure 61).
Thyroid myopathy with enlarged muscle bellies is
seen well in a coronal view but can also be appreciat-
ed in the axial and saggital views. Enlarged muscles
can also be seen in pseudo tumor which involves all
of the muscle and tendon in contrast to thyroid
myopathy that spares the tendon. Myositis is also Figure 59
readily observed as muscle enlargement. These sub- A Axial CT
B Axial MRI - T1 weighted image
tle diagnostic differences can be detected on a com-
plete MRI study.

52
Surgical anatomy

A B

Figure 60
A Normal T1 weighted coronal MRI B Abnormal T1 weighted coronal MRI showing enlarged
inferior recti (dark area).

A B

Figure 61
A Normal T2 weighted axial MRI C Normal T1 weighted saggital MRI
B T1 weighted axial MRI with diminished signal from fat.
Note the enlarged muscle bellies.

53
Chapter 2

While coronal and saggital views are effective the case of strabismus surgery, with a smaller eye. In
for finding muscle belly enlargement, the axial view clinical practice, the most important advantages of
is best for identifying a slipped or lost muscle. using these measurements seem to be consistency and
Because of the anatomic characteristics of the rectus the ability to do the largest recession without crip-
muscles, the medial rectus is the only muscle likely to pling the medial rectus by placing the new insertion
undergo sufficient slippage to present a clinical chal- too far posteriorly.
lenge in finding the muscle. Studies of the globe in neonates and infants
Recent description of the muscle pulleys, partic- indicate that the posterior aspect of the globe is rela-
ularly those related to the horizontal recti, has tively hypodeveloped compared to the anterior
prompted Demer and associates to stress the value of aspect. This means that recession of the medial rectus
imaging for the diagnosis and treatment planning of a in a newborn could put the new insertion site behind
variety of strabismus entities. These include: the equator, even in cases where as little as 3 mm of
incomitant strabismus, A and V patterns, Brown recession were done. Therefore, it has been advocat-
syndrome, heavy eye in high myopia, and others. ed that surgery should not be done on very young
The strabismus surgeon now employs imaging infants. Surgery on infants as young as two or three
on a selective basis. Some deterrents to routine use of months has been reported, but is not something that
imaging include: high cost, inability to use in young could be considered routine or advisable based on
children, lack of need in many cases, and, of course, anatomic studies. In addition, there is good evidence
habit. It is likely, in the future, that techniques will that four months may be the earliest age that congen-
improve and costs will come down making imaging a ital esotropia can be diagnosed with confidence.
more frequently-utilized tool for strabismus manage- Surgery on infants with infantile esotropia between
ment. Ultrasound in the A or B scan mode can be the fourth and sixth month is now common and is
employed as an alternative method for orbital and safe. The axial length of the typical eye at this age is
extraocular muscle imaging. This technique is office 19.5 mm. Such eyes are entirely suitable for surgery
based and less expensive but is more difficult to inter- consisting of bimedial rectus recession to a point
pret. approximately 9.5 mm from the limbus. A safe lower
age limit for surgery in cases of congenital infantile
Growth of eye from birth esotropia with no other contraindications is four
through childhood months. Surgery for congenital esotropia by six
The eye undergoes significant growth between months of age is now common and is shown in Figure
the neonatal period and adulthood (Figure 62). Study 63.
of this growth in vivo is made possible by the use of Nanophthalmos describes an otherwise normal-
accurate, quick, and reliable A-scan biometry. The A- ly functioning eye but with a shorter anteroposterior
scan biometer, used principally for intraocular lens diameter. These eyes have increased scleral thickness
calculations in adults, has been applied to children to but decreased rigidity and are subject to retinal
obtain measurements of the anteroposterior diameter. detachment. These eyes also have hyperopia and an
Gilles first used these measurements which he com- increased incidence of glaucoma.
bined with corneal diameter measurements and meas- High myopia results in a significant increase in
urements of the medial rectus insertion site to arrive the anteroposterior diameter. Anteroposterior diame-
at a more scientific formula for recession of the medi- ters as long as 27 mm in a six-year-old child with
al recti. Kushner found an inverse relationship -11.00 D of myopia have been measured and some
between axial length and response (prism diopter adults have axial length measurements greater than
change per millimeter of surgery) in esotropic 30 mm. This can lead to intermuscular membrane
patients. This finding is expected since the maximum rupture, pulley displacement, and muscle slip causing
torque can be obtained with a smaller gear or, in eso - hypotropia or heavy eye.

54
Surgical anatomy

Figure 62
A One-week-old child, anterior-posterior diameter 17+ mm D One-year-old child, anterior-posterior diameter 20+ mm
B Three-month-old child, anterior-posterior diameter 18+ E Two-year-old child,anterior-posterior diameter 21+ mm.
mm F Three-year-old child, anterior-posterior diameter 22+ mm
C Four-month-old child, anterior-posterior diameter 19.5+ G Five-year-old child, anterior-posterior diameter 23+ mm
mm

55
Chapter 2

Sclera
The thickness of the sclera varies according to
A location (Figure 64).
1. At the limbus, the sclera is 0.8 mm thick.
2. Anterior to the rectus muscle insertions, it is
0.6 mm thick.
3. Posterior to the rectus muscle insertions, it is
0.3 mm thick.
4. At the equator, it is 0.5 to 0.8 mm thick.
5. At the posterior pole, it is greater than 1 mm
thick. The area of greatest surgical activity
for the extraocular muscle surgeon coincides
with the thinnest area of the sclera.
Care must be exercised when placing a needle
into the sclera (Figure 65). A reverse cutting needle
should be used only while exercising extreme caution
because such a needle may be as thick as or thicker
B than the sclera into which it is inserted. This could
lead to scleral perforation, an event that undoubtedly
occurs more often than is suspected or reported.
Fortunately, most cases of inadvertent scleral perfora-
tion heal without incident. If such a cutting needle is
used, it should be very fine (preferably less than 0.3
mm), if possible, and it should be inserted carefully
with the top of the needle seen through the superficial
sclera at all times. For added safety, the cutting edge
can be directed sideways so that it cuts along the scle-
ral lamellae rather than into the eye, as shown. A
curved cutting needle is less likely to perforate the
sclera than a reverse cutting needle, but the curved
cutting needle is prone to cut itself out of the sclera
unless an excessively deep bite is taken.
C A much safer needle to use is the spatula design.
With such a needle, only the tip and sides are cutting
edges. The sclera is displaced upward and downward
away from the body of the needle and is cut laterally
and ahead of the needle. This action makes the com-
plication of scleral perforation less likely to occur
with spatula needles than with cutting needles. The
spatula needles widest dimension should remain par-
allel to the scleral surface. Needles with a wire diam-
eter of .203 mm are both sufficiently strong and deli-
cate enough to be inserted safely.
A keystone spatula, with cutting tip up, is safe
Figure 63 but can cut out of sclera. A keystone spatula, cutting
A Six-month-old with 50 diopters congenital esotropia tip down, produces a longer track but can also cut in
preoperatively. to the eye. A hexagonal spatula or neutral tip needle
B Same patient immediately after surgery in the operating
room. Both medial recti were recessed to a point 10 mm must be guided to stay at mid-scleral level.
from the limbus. The axial length was 19.5 mm in each The sclera is white and opaque when fully
eye. hydrated. If this tissue becomes dried out, it becomes
C Same patient at age 1 year. dark amber-colored and translucent. Re-hydration
rapidly restores the opaque whiteness of the scleral
tissue.

56
Surgical anatomy

A B

Figure 64
A The sclera varies in thickness according to location B The sclera is thinnest, 0.3 mm, posterior to the rectus
muscle insertion

A B

D E

Figure 65
A Keystone spatula, cutting tip down D Reverse cutting - tends to cut in - can be placed
B Keystone spatula, cutting tip up sideways
C Hexagonal spatula, neutral cutting tip E Curved cutting - tends to be cut out

57
58
3
Parasurgical procedures
and preparation

Overview
The instruments required for extraocular mus- surgery. In cases where the health of a patient could
cle surgery are simple and relatively few. As with any be compromised by outpatient surgery, the patient
type of surgery, however, the surgeon must have can be admitted the day before surgery. Occasionally,
available all instruments required for a particular pro- a patient requires admission on the night of surgery
cedure, and these instruments must be in good work- on an unscheduled basis because of excessive vomit-
ing order. ing, breathing difficulties, or some other complication
Anesthesia suitable for extraocular muscle sur- which may be unrelated to the surgery itself.
gery varies according to the patient's individual The advent of mandated outpatient strabismus
requirements and the surgeon's personal preference. surgery requires that the surgeon and staff, including
Children always require general anesthesia with the operating room and recovery nurses and anesthe-
endotracheal intubation or with ketamine dissociation sia staff, participate in thorough preoperative educa-
which can be used with or without endotracheal intu- tion. This includes the family and to some degree the
bation. Insufflation anesthesia, which leaves the patient, including both the understanding child and
patient's airway unguarded except by the patients the adult. The family and/or patient should be made
own response, has been replaced in most cases by to understand that all liquids and solids by mouth
safer techniques which guard the airway. The gener- must be withheld for a period of up to 8 hours before
al anesthetic agent or agents used for children or the scheduled time of surgery. The family and/or
adults are usually determined by the anesthesiologist. patient should be assisted in obtaining necessary pre-
Cooperative adults may be operated on successfully operative laboratory tests before the day of surgery.
with local anesthesia, and a few surgeons have used In the past, this has consisted of determination of
topical anesthesia for extraocular muscle surgery in hemoglobin or hematocrit, but this is now considered
carefully selected patients. unnecessary in a healthy child. Further blood testing
The lids and face around both eyes must be is rarely required. A urinalysis is not required. Most
washed and properly draped and the operative field adult patients over 55 years of age require an electro-
freed of clutter to prepare the patient for surgery. cardiogram (ECG), which is usually performed in the
Antibiotic drops or ointment, often with steroid, are holding area just before surgery. In selected cases of
used postoperatively by nearly all strabismus sur- adult patients and less often in children with a history
geons. Infection after strabismus surgery is not com- of lung or breathing difficulties, a chest x-ray study is
mon, and rarely serious, and reaction to surgery tends required. Patients taking anticoagulants should be
to be mild. However, the use of postoperative antibi- advised to consult their primary physicians regarding
otics often with added corticosteroid is still consid- a safe time to stop and then restart this medicine.
ered worthwhile by many surgeons. A patch may be Likewise, diabetics or patients with other significant
used according to the surgeon's preference, but is usu- health issues must be identified and given proper
ally not necessary. advice, usually by their primary care physician.
Surgery is done on an outpatient basis, with A patient who lives fairly close to the facility
both children and adults arriving at the hospital on the where the surgery is performed may leave from home
morning of surgery and leaving several hours after and return home on the day of surgery. In cases

59
Chapter 3

where the patient lives farther away, i.e., more than The main purpose of the immediate preoperative
two hours by car, it may be more convenient for the examination, in my opinion, is to confirm that the sur-
patient and family to stay the night before surgery in geon has accurate information so that the proper sur-
a hotel, motel, with family living locally, or in a hos- gery will be performed. If the physical status mili-
pital-based housing facility nearby. Likewise, for tates against a safe surgical experience, surgery
comfort and safety, these patients should stay near the should be canceled and rescheduled. In cases where
hospital on the night of surgery. These patients may there is a question about a child being ill before the
be examined on the day after surgery. Other patients day scheduled for surgery, parents are advised to call
needing same day adjustment of an adjustable suture a day or two ahead of surgery and to consult their
can be retained in a short stay hospital unit. A next- local doctor about the advisability of proceeding. The
day examination is an absolute requirement if an combined surgeon-anesthesiologist history and phys-
adjustable suture must be adjusted on the morning ical examination done immediately preoperatively
after surgery but it is optional in most other cases. ensures that the patient is sufficiently healthy to
From a practical standpoint, it is necessary to undergo surgery. A sample preoperative physical
stress to patients the importance of arriving at the examination is shown in Figure 1.
hospital at the appointed time in order to avoid a shut-
down of the operating room for lack of a patient. At Consent for strabismus
the same time, it is necessary to explain that because surgery
of occasional unavoidable delays, the surgery may Before strabismus surgery is begun, appropri-
not start at the scheduled time. It is essential to pro- ate informed consent must be obtained from the
vide the family additional support during the outpa- patient or from a parent or legal guardian. A standard
tient surgery process because both the patients and operative consent form is available in most surgical
their families tend to be less comfortable as outpa- facilities. A sample of this form is shown in Figure 2.
tients compared to in-patients. In addition to the standard consent form with the
usual disclaimers used for any type of surgery, it is
Physical examination necessary to advise patients undergoing strabismus
The physical examination is simple and is usu- surgery of the following complications unique to stra-
ally completed just before surgery. However, to expe- bismus surgery:
dite the flow of outpatient surgery, it may be com-
pleted in the clinic or office up to 30 days* before Diplopia. Patients should be told they might see dou-
scheduled surgery. Likewise, laboratory tests are ble at some time after surgery. This can occur as soon
valid for 30 days*. No matter when the physical as the patient opens his/her eyes after surgery.
examination takes place, the patient's temperature is Diplopia can even be considered a favorable sign in
recorded just before surgery. In addition, at this time, patients with fusion potential. In case of an incomi-
the anesthesiologist performs auscultation of the tant deviation, a patient may be able to find double
lungs and the heart and reviews the patient's current vision looking in one direction, but also may be able
and past medications and the pertinent anesthesia his- to eliminate it looking in another. Patients are told
tory. not to be alarmed if diplopia occurs. They should be
The preoperative history obtained by the sur- told that it either goes away or if not, it can be man-
geon or team includes inquiry about upper respirato- aged successfully in nearly every case.
ry or breathing difficulties, cardiac difficulties, fever,
ear infection, bleeding tendencies, and prior anesthe- Loss of vision. Loss of vision after strabismus sur-
sia difficulties (especially family history of malignant gery can occur, but it is rare! For example, this can
hyperthermia). Any medicine used, including aspirin, occur if a needle is placed too deeply, passing through
should be recorded and made known to the surgeon. the retina and producing a vitreous hemorrhage which
Drug allergies should be noted. The history continues can clear with time, or it can cause retinal detach-
with a review of systems. Physical examination ment. Although scleral-retinal perforation may occur
includes evaluation of the heart and lungs and an in as many as 1% of strabismus cases, significant
overall observation of the patient. complications from this cause are rare. Infection pro-
The eye findings that were recorded in the clin- ducing endophthalmitis can also cause loss of vision.
ic chart at the time surgery was scheduled should be Fortunately, this too is extremely rare. Anterior seg-
reviewed, and the patient's current motility should be ment ischemia causing cataract can cause variable
compared to these findings. If there is a major dis- reduction in vision.
crepancy between these findings, it may be prudent to
cancel surgery, although this action is rarely taken.

* Time limits may vary at different facilities.

60
Parasurgical procedures and preparation

Figure 1

61
Chapter 3

Figure 2

62
Parasurgical procedures and preparation

Need for reoperation. The need for a reoperation scissors, fine forceps, muscle hooks of various sizes,
after strabismus surgery is not really a complication retractors, needle holders, a caliper, and something to
by itself. In most cases, before doing strabismus sur- provide cautery. An open flame and a probe project-
gery, the surgeon can give the patient an approximate ing from a metal ball are used in many developing
percentage figure for the need for reoperation. For countries to achieve cautery. Although a variety of
example, the surgeon can tell the patient/family that specialized instruments have been introduced over
in spite of making a 100% effort to straighten the the years, these basic instruments have remained
eyes, there is a (10%, 20%, 30%, etc.) likelihood of essentially unchanged in number and design for a
the need for reoperation. In the case of congenital hundred years or more.
esotropia, this percentage is between 5% and 20%. In In response to finer sutures and needles, better
cases where prior surgery has been performed, when understanding of anatomy, and more widespread use
the strabismus is complicated, or after injury, need for of magnification, several modifications to the basic
reoperation may be as high as 50%. A reoperation instruments for strabismus surgery have been made.
may be necessary, even in a case where everything These instruments are now available from Katena
goes exactly as planned, because a totally predictable Products, Inc. (Figure 4). These include the
response is not possible in every case. On the other Lieberman speculum in an adult and pediatric size;
hand, in cases of a slipped or lost muscle, excessive and the Helveston Barbie retractor in three sizes,
hemorrhage, fat exposure, etc., reoperation may be standard, big, and great big, used in place of the
necessary where things did not go as planned. bulkier Desmarres retractors. Also newly modified
A separate consent obtained by the anesthesiol- are three muscle hooks including the small, right
ogist can include information about the chance of angle teaser hook to be used in place of the heavier
breathing difficulties, vomiting, sore throat, or even curved Stevens hook; three sizes of the standard mus-
of heart attack or death! cle hook with a finer hook end to be used in place of
the bulkier Jameson hook; and two sizes of a sharp,
Instruments used in pointed finder hook to be used very carefully as a
strabismus surgery combination muscle hook and dissector. A curved
The complete instrument assortment for stra- caliper is a modification of an earlier instrument
bismus surgery is shown as assembled on the instru- developed by William Scott. This newer caliper is
ment stand (Figure 3). Not every instrument is used both finer at the tip which also has a marking point
in each case, but ideally, all should be available each and has a longer handle making it easier to use. A
time strabismus surgery is done. In case the complete modification of the Moody locking Castroviejo
set is not available, the minimum instruments for stra- includes a curved, heavier handle and a more durable
bismus surgery are the following: lid speculum, fine sliding lock mechanism.

Figure 3

63
Chapter 3

A D

B E

C F

Figure 4 Helveston instruments


A Teaser hook D Barbie retractor (3 sizes)
B Finder hook E Scleral ruler (modified from William Scott)
C Fine muscle hooks F Moody-Castroviejo curved locking forceps (with slider)

Other specialized instruments for strabismus or tenotomy whose only effect was to weaken the
surgery that have not been shown in the complete sur- muscle. Simply cutting the muscle like this frequent-
gery set are used according to the individual sur- ly produced overcorrection that was difficult or
geons preference. These include various muscle impossible to reverse. As a result, the initial wave of
resection clamps; the superior oblique tendon tucker enthusiasm for strabismus surgery waned.
in several sizes; muscle hooks with a thin metallic By the second half of the nineteenth century,
shield to guard against too deep needle placement; heavy silk sutures about the size of current 4-0 or 5-0
the heavy Green muscle hook especially used by calibre were employed to control weakening and also
some surgeons who perform the cul-de-sac incision; a for muscle shortening or strengthening. These silk
hook with a double foot to aid suture placement; and sutures were often waxed at the time of surgery to
more. help ease the passage through tissue. The re-usable
The size and style of forceps depends on the needles for introducing this suture were large, made
surgeons preference. In general, two sizes include of round wire, and had an eye to thread the suture.
the heavier size with 0.5 mm teeth and the more del- Because of the bulk of these needles compared to the
icate forceps with 0.12 mm teeth, especially useful thickness (thinness) of the sclera, sutures secured the
for grasping the conjunctiva. The design of the teeth, muscle to overlying Tenon's capsule and conjunctiva
especially those found on the 0.5 mm forceps, vary in with the knots tied externally. Muscles were not
their effect on tissue grasping and tissue tearing. The anchored to the sclera as done today. These silk
Pearse forceps features a half circle cut out on each sutures eventually had to be removed to avoid infec-
arm of the forceps that have square tips. This design tion or excess reaction.
is easier on tissue. There is also a wide array of nee-
dle holders either locking or non-locking with jaws of Animal product absorbable suture
varying size and shape suitable for the needles used Absorbable, catgut suture that is actually pro-
according to the surgeons preference. duced from sheep intestine was introduced for stra-
bismus surgery around the turn of the twentieth cen-
Sutures and needles for tury. This suture was used widely until the early
strabismus surgery 1970's. Catgut suture offered a definite advantage
over silk by being absorbable. Disadvantages of
Overview, historical perspective catgut suture are that it lacks strength and uniformity.
These sutures also produce significant inflammatory
Nonabsorbable suture - early use reaction. In an attempt to remedy some of these prob-
Strabismus surgery, introduced by Dieffenbach lems, collagen suture was introduced in the 1960's.
in 1839, was done without suture. At the outset, tech- Though still an animal product absorbable suture and
niques were limited to partial or complete myotomy no stronger than catgut, collagen promised some

64
Parasurgical procedures and preparation

improvement over catgut. Collagen suture is formed A small drawback of braided synthetic
by an extrusion of homogenized, pooled beef fascia absorbable suture is a tendency for the suture to
and is 100% collagen, making it smooth, conforma- engage tissue and drag this tissue along with the
ble, and easy to handle. Collagen suture also is easy suture (Figure 5). This can lead to premature knotting
to tie, producing a secure knot. Theoretically, the and inaccurate tissue apposition. When using this
pooled fascia making up collagen suture should result synthetic suture, it is important for the surgeon to be
in reduced antigenicity and therefore reduced inflam- aware of this and to avoid tissue drag while advanc-
matory reaction making it superior to catgut. On the ing the suture. With all of the advantages offered by
contrary, reaction with collagen is similar to that seen this new suture, this small annoyance is just that,
with catgut. Mild to moderate inflammatory reaction small and manageable. Another thing to remember
occurs in about one case in five and severe suture with this synthetic absorbable suture is that it does not
granuloma occurs in about one percent. An addition-
al drawback is that when using either catgut or colla-
gen, anyone but the most expert surgeon can expect to
break at least one suture per strabismus surgical pro-
cedure!
A
Synthetic absorbable suture
Beginning in the 1970's, synthetic absorbable
suture became available for strabismus surgery. This
material is a polymer of sugar and is called polygly-
colic acid or polyglactin 910, the latter also known as
Vicryl. This uniform, braided suture is coated with a
material similar to that used for making the suture
itself. This coating is added to smooth the suture
making for easier passage through tissue. For either
recession or resection of a muscle, 6-0 suture is suf-
ficiently strong and is the choice of most surgeons.
For closure of the conjunctiva, 8-0 suture may be
used. Some surgeons prefer to use an 8-0 collagen for B
this purpose because this suture dissolves readily and
strength is not an issue for this suture application.
In addition to being both strong and uniform,
synthetic absorbable suture is less likely to cause tis-
sue reaction as seen with animal product sutures. The
incidence of tissue reaction of any kind with synthet-
ic absorbable suture is on the order of one percent or
less. These sutures retain holding properties for 14 to
21 days, a time more than sufficient to ensure secure
healing of the extraocular muscle to sclera, something
which actually takes place in a few days. The syn-
thetic absorbable suture absorbs completely in about
three months.
In a recent survey of experienced strabismus C
surgeons, the introduction of synthetic absorbable
suture was near the top in importance of all of the
innovations for strabismus management in the past
half century. Several of those surgeons considered
the introduction of this suture to be the most impor-
tant strabismus treatment advance in this period. It is
likely that the re-introduction of adjustable sutures,
another of the strabismus related top ten events of
the past half century, was made possible largely Figure 5
because of synthetic absorbable suture. This suture is A The rough braided surface of braided Vicryl can lead to
tissue grab as it passes through Tenons capsule
strong enough to allow the manipulation required at B Comparison of 1) collagen, 2) uncoated, undyed Vicryl,
adjustment while also being absorbable and minimal- 3) coated, dyed Vicryl
ly reactive. C Poor muscle scleral union due to premature knotting

65
Chapter 3

dissolve unless buried. Exposed knots become stiff muscle to sclera? To answer these questions, Coats
and can be irritating. It is sometimes necessary to cut and Paysse placed 6-0 Vicryl sutures into human bank
off the knots of exposed 8-0 sutures used to close con- sclera with the suture track at various depths and
junctiva. lengths and then measured the force necessary to pull
Nonabsorbable suture the suture free from sclera. The results showed that
suture placed in a track 1.5 mm or longer at a depth
Nonabsorbable suture such as 5-0 or 6-0 nylon
of at least 0.2 mm required in excess of 200 gm force
or Dacron which causes minimal reaction and is supe-
to cause the suture track to fail. This is a greater force
rior to silk may be used for tucking procedures car-
than can be expected in the physiologic state, indicat-
ried out on the superior oblique tendon or rectus mus-
ing that this manner of suture placement in sclera is
cles. This type of suture is also useful for rectus mus-
both safe and effective (Figure 7).
cle union as done with the Jensen procedure or more
Some surgeons prefer the crossed swords
recently that described by Foster and Buckley for the
needle placement as described by Parks (Figure 8).
enhancement of rectus muscle transfer. The posterior
With this technique, the needles are passed through
fixation suture (retro-equatorial myopexy) or faden
sclera for approximately 5.0 mm or longer. These
operation is also a place where non-absorbable
sutures are useful.
Black silk sutures size 4-0 or 5-0 are useful for
traction during surgery. They are placed temporarily
A
near the limbus to pull the eye in a given direction to
facilitate exposure. Another use of black silk traction
sutures is to pull and then anchor the eye in an exag-
gerated direction for a few days after surgery. This
maneuver is designed to oppose early postoperative
healing that could tend to negate some or all of the
weakening effects of surgery. These silk sutures are B wire diameter
usually inexpensive and have large needles which (before fabrication)
must be inserted into episclera with care, but which C
easily pass through the lids when necessary. H I
D
Needles
The anatomy of an ophthalmic needle is E F
shown in Figure 6. The relationship of needle size
and configuration and anatomy of the sclera is dis- G
cussed in Chapter 2. The choice of a needle for stra-
bismus surgery is based in part on surgeon's prefer- Figure 6
ence, availability of needle and suture combinations, A Various dimensions F Keystone - down cutting
and the unique requirements of a given procedure. B Round tip
For recession and resection, a spatula needle with a C Cutting G Hexagon - neutral tip
D Reverse cutting H Parallel
wire diameter of .203 mm is suitable. The surgeon E Keystone - up cutting tip I Cobra head
should be aware that the configuration of the tip of the
needle will influence the path of the needle while
passing through sclera. If the tip bevels downward,
the needle will tend to go deeper into and even
through sclera. A neutral tip will go where the needle
is directed and a tip beveled upward will have a ten-
dency to cut out and therefore must be continually
directed slightly downward to stay in sclera. Any of
these needle types is acceptable, but for safety, it is
necessary for the surgeon to be aware of the needle
configuration. If there is any question, the surgeon
should examine the needle tip with magnification
before the surgery is begun.

Needle placement in sclera


How deeply should the needle (suture) be
placed in sclera and how long should the suture track
be placed in sclera to ensure secure attachment of the Figure 7

66
Parasurgical procedures and preparation

two long shallow bites do not provide any more secu- about the safety of using succinylcholine, it should
rity for muscle attachment to the globe once the not be used. As a substitute, pancuronium, or other
sutures are tied, but this technique does allow the nondepolarizing muscle relaxant, may be used.
muscle to remain securely at the point of intended In cases scheduled for general anesthesia
recession against the sclera even before the suture is where a family history of malignant hyperthermia in
tied because of friction in the suture tunnel. When uncovered, pre-treatment with dantrolene is required.
using the crossed swords technique, the first needle Study of phenylketopyruvate serum levels can be use-
is left in place until the second needle is inserted into ful in predicting susceptibility to malignant hyper-
sclera. Each needle is then advanced in turn until
both needles just clear sclera. After this, the suture on
each needle is advanced in turn. This maneuver is
carried out to avoid having the sharp side of the spat-
ula needle cut the other end of the suture being
advanced. A needle with a down directed tip makes
this maneuver easier to perform, but extra care is
required to prevent the needle from going too deep.
Perforation of sclera by a needle placed too
deeply probably occurs more frequently than is sus-
pected. Morris, et. al. in a prospective study found
one perforation in 67 patients, 100 eyes. This is like-
ly to be a reliable statistic (1%) even for experienced
surgeons. A too deep suture rarely causes a prob-
lem. Moreover, it is not usual practice for a surgeon
to dilate the pupil and look at the retina over the site
of muscle reattachment, meaning that most of these
occurrences probably go undiscovered and unreport-
ed. This may be for the best since overzealous Figure 8
attempts at treating these mostly innocuous retinal The crossed swords technique of Parks.
perforations has resulted in serious complications
including loss of the eye (Figure 9).

Anesthesia for strabismus


surgery
The choice of anesthesia (general, dissociative,
local, or topical) for strabismus surgery is influenced
by age of the patient, the wishes of the patient, the
requirements of the patients strabismus condition,
and finally by the experience or preference of the sur-
geon. The popularity of adjustable sutures, often
adjusted during surgery, has made the use of topical
and local anesthesia more common in recent years. A
few surgeons use general anesthesia in cases with
adjustable suture, reinstituting a brief general anes-
thetic for adjustment. If general anesthesia is the
choice, the anesthesiologist decides on the specific
agent or agents to be used, as well as the amount and
type of premedication. For patients using echothio-
phate iodide (phospholine iodide), the use of suc-
cinylcholine is definitely contraindicated. A patient
who has been treated with echothiophate iodide can
retain low blood levels of pseudocholinesterase for
weeks or even months after discontinuing the medi-
cine. In the presence of low blood pseudo-
cholinesterase levels, succinylcholine causes pro- Figure 9
Fine ophthalmic needles should be advanced along their
longed apnea that may require the patient to be assist- curve and not forced through tissue or used excessively to
ed by a respirator for several hours after surgery to lift tissue. Failure to observe this can result in a bent nee-
ensure proper breathing. If there is any question dle.

67
Chapter 3

thermia in a patient with a questionable family histo- gery. There is also less anesthesia hangover and less
ry. When a patient with malignant hyperthermia or a postoperative analgesic agent may be needed. The
suspicion of this condition is to be given general induction dose is 2 to 3 mg/kg. Anesthesia is main-
anesthesia, in addition to pre-treating the patient with tained with an IV drip titrated at approximately 200
dantrolene, the anesthesia machine is flushed with mg/kg/min. Low doses of nitrous oxide or halothane
oxygen for 24 hours to rid the machine of all traces of may be used to supplement anesthesia.
halothane. The preferred general anesthetic regimen When an adjustable suture is used and adjust-
for patients with malignant hyperthermia is fentanyl, ment is contemplated for the afternoon of surgery,
nitrous oxide, and a muscle relaxant. preoperative and postoperative narcotics should be
withheld, used in a limited fashion, or reversed after
General anesthesia the operative procedure using naloxone (Narcan).
Most immature patients (younger than mid- With outpatient surgery now routine and nar-
teens) require general anesthesia for extraocular mus- cotics and barbiturates withheld from children preop-
cle surgery. This anesthetic is administered through eratively, a dramatic reduction in postoperative vom-
an endotracheal tube or with a laryngeal cuff with the iting is noted. The advantage of being able to use a
anesthetic agent(s) and oxygen delivered directly to slightly reduced amount of general anesthetic agent
the lungs. The agent most commonly used for gener- after pre-medication with narcotics is outweighed by
al anesthesia is halothane (Fluothane). Other agents the fact that after a short procedure, a heavily pre-
such as fluroxene (Fluoromar); cyclopropane; medicated patient may exhibit prolonged drowsiness,
methoxyflurane (Penthrane); and a combination of not from the general anesthetic agent, but from the
nitrous oxide, barbiturate, and narcotic may be used preoperative medications.
but have no advantage over halothane. Thiopental General anesthesia allows the surgeon more
sodium (Pentothal) given intravenously or nitrous freedom in manipulation of the muscles and accurate
oxide given by mask is the most common agent used interpretation of passive ductions. Therefore, many
for induction before intubation. Versed 0.5 mg/kg surgeons prefer general anesthesia for all strabismus
may be used as a pre-anesthetic calming drug in surgery. As with general anesthesia used for any type
excitable younger children between ages 1 and 7 of surgery, patients undergoing strabismus surgery
years. It is given in a grape-flavored liquid in the out- should be monitored constantly by the anesthesiolo-
patient holding area. Open-drop ether, which had gist in order to diagnose immediately emergencies
been used for induction of anesthesia for infants, is such as arrhythmia, hypoxia, bradycardia associated
now of historic interest. Ether has a wide margin of with the oculocardiac reflex, or cardiac arrest.
safety, but postoperative vomiting is frequent. When bradycardia from the oculocardiac reflex
Preoperative medication for infants should be occurs, all tension on the muscle should stop immedi-
limited to a moderate dose of atropine given intra- ately, and the muscle should not be placed under ten-
muscularly. The dose for preoperative atropine is sion again until the heart rate returns to normal. It is
0.01 mg/kg, with a minimum of 0.1 mg and a maxi- not necessary to remove the muscle hook from behind
mum of 0.4 mg. Older children and adults having the muscle's insertion, but the surgeon must relax all
general anesthesia may require narcotics and, in some pressure on the muscle insertion. If repeated muscle
cases, barbiturates in addition to atropine. The doses stimulation causes further bradycardia, the patient
should be selected on an individual basis. should be given intravenous atropine by the anesthe-
Preoperative narcotic is associated with a high- siologist, the dose is usually the maximum 0.4 mg
er incidence of postoperative nausea and vomiting. intravenously. The use of atropine in adults to block
With the advent of outpatient surgery, pre-medication the oculocardiac reflex increases the likelihood of
is omitted except in cases where Versed is used. cardiac arrhythmia such as bigeminy. Bradycardia
Preoperative medication with droperidol 0.075 mg/kg persisting after atropine has been injected intra-
has been said to reduce postoperative vomiting from venously may be treated with a retrobulbar injection
60% to 16% when given intravenously before muscle of 1 to 3 ml of 1% or 2% lidocaine (Xylocaine).
manipulation. This medication does not prolong the Bradycardia from the oculocardiac reflex rarely dis-
patient's recovery to full alertness or the time in the rupts the normal conduct of surgery. If it does occur,
recovery room. Outpatient strabismus surgery can be it requires just a few seconds pause. With real-time,
performed safely with only versed for pre-medication accurate monitoring, including audible pulse record-
and without other prophylaxis for postoperative vom- ing, the surgeon should be able to recognize the earli-
iting. Even with this minimalist routine, postopera- est signs of bradycardia and reduce the pull on the
tive vomiting occurs rarely. muscle. This usually leads to restoration of the
A new agent for general anesthesia is Propofal patient's normal heart rate in just a few seconds.
(Diprivan), a drug which is administered intravenous- Cardiac arrest is treated with ventilation and closed
ly. Propofal has the advantage of reducing the inci- chest heart massage both begun immediately. If car-
dence of nausea and vomiting after eye muscle sur- diac contraction does not begin after several minutes,

68
Parasurgical procedures and preparation

3 to 5 ml of intravenous epinephrine 1:10,000 may be ization of the needle before injection. Ketamine anes-
given. I have no personal experience with this or thesia is used widely, usually with other appropriate
other more radical measures. agents, in developing countries for strabismus surgery
because of its wide margin of safety.
Dissociative anesthesia Anesthesiologists skilled in the use of ketamine can
Ketamine, a dissociative anesthetic, has been administer this drug effectively and apparently with
used for a variety of ophthalmic procedures, includ- few of the drawbacks listed here.
ing strabismus surgery. With ketamine, the patient
has no cognizance of pain because the drug causes a
Local anesthesia
dissociation between the painful stimulus and any Perilimbal anesthesia
awareness of the stimulus. Involuntary movements of
Either 1% or 2% lidocaine (Xylocaine), with or
all parts of the body, including the eyes, do persist
without epinephrine 1:100,000 added, provides satis-
with ketamine, and tonus of the extraocular muscles
factory local anesthesia for strabismus surgery in
remains. Therefore, the eye must be stabilized with
cooperative teenagers and adults (Figure 10). For
traction sutures and the surgeon must be constantly on
perilimbal anesthesia, between 1 and 3 ml of the
the alert for unexpected ocular movements. The per-
agent is injected into the subconjunctival space for
sistent muscle tonus also makes interpretation of pas-
360 degrees around the limbus. This is followed by 1
sive ductions less reliable. Patients under ketamine
minute of gentle massage with the fingers through a
anesthesia also secrete saliva freely, which requires
4" x 4" gauze pad over the closed eye. Surgery may
suction during the procedure. The dose of ketamine
then begin. A variety of other techniques for anes-
is titrated to effect and is often given with other anal-
thetizing the anterior part of the eye to allow safe and
gesics or sedatives.
effective eye muscle surgery including peribulbar
In older children and adults, the combination of
anesthesia can be employed. In all cases where local
prolonged drowsiness, disturbing dreams, and hallu-
or topical anesthesia is used, oxygen is provided con-
cinations is a significant drawback to using ketamine.
tinuously via nasal catheter under the drapes. With
The disadvantages of ketamine may outweigh its
oxygen used this way, care should be exercised to
advantages for strabismus surgery. However, keta-
keep open flame or red hot thermal cautery away
mine's unique properties make it an ideal agent when
from the flowing oxygen to avoid fire. In addition, an
anesthesia is required for injection of Botox in infants
intravenous line is kept open with a normal saline
and children young enough to avoid the side effects.
drip and constant ECG monitoring is maintained. An
Persistent firing of the motor end-plates, which can be
anesthesiologist may be in attendance and may use
detected by electromyography, allows accurate local-
intravenous analgesics as needed.

A B

C D

Figure 10
A A small subconjunctival bleb is raised C Up to 3 ml of anesthesia has been injected
B Injecting into the previous bleb, the limbus is ringed D Gentle massage is carried out

69
Chapter 3

Retrobulbar anesthesia Topical anesthesia


For retrobulbar anesthesia, 2 or 3 ml of 1% or
Surgery may be performed on the extraocular
2% lidocaine (Xylocaine), with or without epineph-
muscles of a cooperative adult using only tetracaine
rine 1:100,000 added, is injected into the retrobulbar
or cocaine hydrochloride 4% solution instilled in the
space (into the muscle cone). A 38 mm, 25-gauge
cul-de-sacs and on the cornea before surgery and
needle on a 5 ml syringe is used. The needle tip
repeatedly on the operative site during surgery.
enters through the skin just inside the orbital rim at
Several drops of lidocaine (Xylocaine) 5% may also
the junction of the inferior and lateral orbital rim.
be used topically. Because this technique demands an
The needle is directed slightly medially and superior-
extremely cooperative patient combined with a sur-
ly for nearly the full length of the needle. The agent
geon who uses delicate technique, it has limited appli-
is then injected slowly. Some surgeons inject slowly
cation. With use of topical or local anesthesia, it is
while the needle is entering to push tissue away from
possible to employ cover testing and/or diplopia test-
the tip. One may also retract the plunger to ensure
ing during surgery with the patient either lying on the
there is no backflow of blood before injecting. This
table or sitting up. Results for surgery may be
avoids inadvertently delivering the agent intravenous-
enhanced by this on the table testing which allows
ly. In the rare event of a retrobulbar hemorrhage, sur-
adjustments in the amount of surgery at a time when
gery is postponed.
the surgeon is free to titrate the amount of surgery. It
After approximately 5 minutes, surgery may
is not necessary to block the lids for successful sur-
begin. A successful retrobulbar injection gives satis-
gery.
factory anesthesia to the anterior globe and extraocu-
The use of local and topical anesthesia
lar muscles. However, the patient may experience
demands cooperation by the patient and intense con-
pain deep in the orbit, presumably in the area of the
centration by the surgeon. Local anesthesia can be
ligament of Zinn, when muscles are tugged on, par-
used for both first surgeries and repeat surgeries. In
ticularly during a resection procedure. Patients also
some cases, the opportunity to adjust surgery on the
may experience pain when the insertion of a muscle
table and the fact that the patient avoids the postoper-
is manipulated. Adjustment of a suture can be done
ative discomfort frequently associated with general
several hours after retrobulbar anesthesia. Proper
endotracheal anesthesia makes the extra effort using
timing for adjustment can be determined by observ-
local and topical anesthesia worthwhile.
ing full rotations of the eye.
The awake patient Preparation of the patient in
The surgeon must exercise greater care when the operating room
performing surgery under any type of local anesthesia The patient should be positioned with the head
with the patient awake. Care must be taken to ensure at the end of the operating table. The surrounding
gentle manipulation and to exert very little traction on area should be free of unnecessary equipment. If gen-
the extraocular muscles because of the deep orbital eral anesthesia is used, connectors should be fash-
pain this maneuver produces at the origin of the mus- ioned so that tubes will not interfere with the surgical
cles. Topical tetracaine or xylocaine 5% anesthetic field. Although the endotracheal tube is well
drops may be placed on the cornea and on the opera- anchored, the surgeon should always warn the anes-
tive site both before initial injection of lidocaine and thesiologist before moving the patients head. After
repeatedly during the procedure. Anesthetic agent anesthesia has been obtained, the area around both
may be injected before the patient's face is prepared eyes should be washed thoroughly. The skin in the
with the surgical scrub and before the surgeon scrubs. area outlined in Figure 11 is washed using Betadine
This interval allows sufficient time for the anesthetic soap or equivalent and water for 1 to 3 minutes fol-
agent to take effect. If needed, additional lidocaine is lowed by a thorough rinse with sterile water. Several
injected around the operative site during the proce- drops of Betadine solution are then placed in the cul-
dure. The conjunctival anesthesia usually wears off de-sacs. This solution is then rinsed from the eyes
sooner than that around the muscle insertion. During with sterile normal saline. An iodine preparation may
the procedure, the patients may also receive the syn- then be painted over the area that has been scrubbed
thetic narcotic agents, fentanyl citrate 2 mg/kg, and and the skin is blotted dry. It is not necessary to trim
diazepam 0.25 mg/kg. The smallest possible amounts the eyelashes. Cloth drapes or self-adhering drapes
of these agents are given to ensure patient alertness, are placed over the nose, forehead, and sides of the
especially when the patient is asked to cooperate dur- head. After this draping is completed, forced duc-
ing adjustment of alignment on the table. tions are carried out in all directions in both eyes. A
self-adhering monocular plastic drape or equivalent is
then placed over the lids with the adhesive applied to
the lids and periocular area (Figure 11).

70
Parasurgical procedures and preparation

A B

C D

Figure 11
A Area of skin washed D Concave head support
B Endotracheal tube firmly anchored E Fully draped operative site using disposable drapes
C Cloth drapes (cloth may also be used).

71
Chapter 3

Figure 12
1) Patient (the most important part); 2) scrub nurse; 3) anesthesiologist; 4) surgeon; 5) assistant; 6) Mayo instrument
stand; 7) back table for drapes, solutions, etc.; 8) viewable monitors; 9) anesthesia machine

Layout of the operating room


and anesthetic apparatus
The layout of the operating room may include real-time information available to surgeon, anesthesi-
the features illustrated in Figure 12. This is a scheme ologist, and nurses. Information that is available
which is common and convenient. includes ECG configuration, pulse, temperature, and
oxygen and carbon dioxide saturation levels. For
Patient monitoring more detailed study, the ECG paper strip may be run.
In the ideal setting, after endotracheal anesthe- The pulse rate is displayed on a digital readout. The
sia has been established, the patient monitor and safe- temperature is constantly displayed on the digital
ty devices are connected (Figure 13). The array is as screen. One of the most effective monitoring capa-
follows: (1) endotracheal tube, (2) mass spectrograph bilities of modern anesthetic machines is the constant
lead, (3) esophageal stethoscope, (4) nasal tempera- monitoring of expired carbon dioxide seen on the
ture probe, (5) ECG leads, (6) heating blanket, (7) anesthesia console (Figure 14).
Doppler arterial flow sensor, (8) sphygmomanometer Many surgeons are more comfortable seated
cuff, and (9) indwelling intravenous catheter. The when operating (Figure 15). A variety of stools are
monitoring display unit situated at the foot of the available. It is important that the surgeon choose one
operating table in clear view of both the surgeon and that he or she can adjust to prevent unnecessary inter-
the anesthesiologist is ideal but not a requirement. ruption of surgery required while an assistant adjusts
This setup when available provides clearly presented, the surgeons stool.

72
Parasurgical procedures and preparation

Figure 13
1 endotracheal tube 6 heating blanket
2 mass spectrograph lead 7 Doppler arterial flow sensor
3 esophageal stethoscope 8 sphygmomanometer cuff
4 nasal temperature probe 9 indwelling intravenous catheter.
5 ECG leads

Figure 14 Figure 15
Console of a modern anesthesia machine Surgeons adjustable stool

73
Chapter 3

Magnification in strabismus Wearing a mask


surgery A mask of paper or cloth should be worn by all
The use of smaller diameter suture material personnel in the operating room. It is disturbing to
and finer, sharper needles has made it preferable, if see a mask worn below the nose. This is a breach of
not necessary, to use magnification for strabismus technique seen all too often in some operating rooms
surgery. Telescopes mounted on glasses frames or on of the world. This lapse of technique should not be
a headband are extremely useful. The magnification tolerated. It defeats the purpose of the surgical mask.
may vary from 2.5X to 4.5X. The limiting factors in
magnification include (1) surgeon's comfort, (2) re-
stricted field size, (3) limited depth of focus, and
(4) need for increased illumination.
For comfort, a properly fitted pair of spectacle
frames with a wide elastic band behind the head con-
necting the temple pieces of the glasses frame works
well (Figure 16). The surgeon soon becomes accus-
tomed to the various restrictive factors associated
with use of a magnifying device while enjoying the
improved view. If the surgeon has presbyopia, he or Figure 16 Operation telescopic loupes
she may choose to place a suitable add low in the
spectacle lens to obtain a wider useful field of vision
when looking at near, but away from the operative
field and around the loupes (Figure 17).

How much magnification is best?


Field size decreases with increase in magnifi-
cation. The trade-off should be arrived at by the sur-
geon through a trial and error method. Working dis-
tance is unique to the particular magnifying instru-
ment used. This should be selected according to the
surgeon's preference, but the working distance should
not be too close. Depth of focus also should be deter- Figure 17
mined by trial and error. Illumination may be Zeiss telescopic loupes mounted on a sturdy spectacle
frame with an elastic head strap
improved by using an overhead operating room light
that is properly adjusted. Some surgeons prefer addi-
tional light supplied by a head-mounted fiberoptic
light (Figure 18).
A few surgeons use a floor-mounted or ceiling-
mounted microscope for strabismus surgery. This
technique provides excellent magnification and illu-
mination; however, with this technique, the surgeon is
even more severely restricted. Those surgeons who
use a microscope for strabismus surgery are strong
advocates for this technique. I suspect that once a
surgeon has used the operation microscope and is rec-
onciled to the trade off, it is difficult to go back to
lower magnification. The optics and illumination of
the operation microscope can be used to obtain video-
tapes of strabismus procedures. During these cases,
the surgeon may elect to use a loupe for magnification
while operating around the microscope. Some sur-
geons who use the technique of anterior ciliary vessel
salvage while recessing or resecting an extraocular
muscle have recommended use of a microscope. Figure 18
A fiber optic head-mounted spot light pro-
vides superior illumination especially when
working in a deep, narrow cavity.

74
Parasurgical procedures and preparation

Outpatient surgery: the day of


surgery
A scheme for outpatient surgery begins with examination. The anesthesiologist also obtains a his-
the patient arriving and checking in at the outpatient tory, performs a physical examination, and obtains
surgery unit. For 8:00 AM surgery, arrival time is consent for the anesthesia. Pre-medication with
between 6:30 and 6:45 AM. The patient may go to Versed may be given in selected children at this time.
the laboratory, if a hemoglobin-hematocrit is to be The patient is taken to the operating room where the
obtained. If done in advance of the day of surgery, surgery is carried out. After surgery the patient is
laboratory results usually remain valid for 30 days in taken to the recovery room and remains there until
an otherwise healthy patient. Depending on the age fully reactive. This takes 20 to 40 minutes or longer.
and health of the patient, the anesthesiologist may The intravenous line is usually discontinued before
choose to waive the hemoglobin determination. The the patient leaves the recovery room. The patient
patient returns to a holding area where the surgeon returns to the outpatient area holding room. The
verifies the ocular motility findings, reviews the sur- patient leaves the outpatient area (for an 8:00 AM
gical plan, obtains operative consent, obtains a perti- case, approximately 2:00 PM) (Figure 19).
nent medical history, and performs a brief physical

Figure 19
1 Check in 6 Postoperative holding area (could be same room as pre-
2 Laboratory, if needed operative holding)
3 Physical examination/consent holding area 7 Discharge
4 Operating room
5 Recovery room (patient could be discharged from recov-
ery room)

75
Chapter 3

Postoperative care of the


patient
A patch may be used postoperatively over one patient to patient, according to the type of anesthesia,
eye, but never over both eyes. Any questionable ben- and depends entirely on the effects of anesthesia.
efit resulting from reduced ocular motility when bilat- When local or topical anesthesia is used in adults,
eral patches are used is far outweighed by the emo- they frequently leave the area in an hour or less.
tional trauma this practice causes. If both eyes have When a general anesthesia is given, children are
been operated on with a single muscle treated in each required to stay for a minimum of 1 to 3 hours before
eye, no patch is used. If a recession-resection has the anesthesiologist discharges them. Before patients
been performed on one eye, a patch may be placed on who have had general anesthesia are discharged, it is
the operated eye for 24 hours and then removed. If prudent to determine that they can retain clear liquids
three or more muscles are involved and both eyes in small amounts.
have been operated on, the eye with greater reaction Patients are seen 1 week and again 8 weeks
may be patched for 24 hours only. A patch for at least postoperatively. At the 8-week visit, the results of
one eye is definitely indicated when the cornea has surgery are usually apparent. In cases where early
been abraded during the course of surgery. In addi- postoperative treatment such as prisms, patching, or
tion to using the patch in cases of corneal other techniques are necessary, the postoperative rou-
drying/abrasion, homatropine 5% or other longer act- tine is individualized according to the patient's needs.
ing cycloplegic drops are placed in the cul-de-sac and Specific postoperative management for individual
sulfacetamide sodium and prednisolone combination cases is discussed in later chapters.
or equivalent drops are used. Ointment is avoided. Less than 1 in 50 patients scheduled for outpa-
As with patching, the use of drops or ointments tient surgery has an unplanned overnight admission,
postoperatively varies from surgeon to surgeon. usually for one of two causes: persistent vomiting or
Some prefer none, whereas others use antibiotic drops breathing difficulties. The incidence of postoperative
or ointment, and still others use antibiotics and vomiting has decreased dramatically in both severity
steroids in combination. An ointment with sodium and frequency with the advent of outpatient surgery.
sulfacetamide and prednisolone combined, once or The most obvious variable is the fact that outpatients
twice a day for 5 to 10 days is a safe choice. Although are not receiving preoperative narcotics. Careful pre-
rare, infection after eye muscle surgery does occur operative evaluation by both the surgeon and the
and the morbidity from use of antibiotics and steroids anesthesiologist identifies most infants and children
is extremely low. Patients also tend to be more com- who have the potential for postoperative breathing
fortable with drops. difficulties because of upper respiratory infection.
Patients, parents, or other family members are When a potential problem is identified during the pre-
contacted immediately after surgery and a report is operative work up, surgery is cancelled or the anes-
given on the patient's response to surgery. This is thesiologist takes special precautions. However, the
done preferably in person or by phone. Within the best precaution is to tell parents if they have any ques-
first 1 or 2 hours after surgery, the patient's ocular tion about their child's health to call the outpatient
motility is checked in the holding area. Patients are facility the day before scheduled outpatient surgery.
discharged routinely 2 to 4 hours after surgery. The A proper decision made at this time could save an
time between the end of surgery and discharge from unnecessary trip to the hospital.
the outpatient postoperative holding area varies from

76
Section 2

Chapter 4: Work up of the strabismus patient

Chapter 5: Diagnostic categories and classification of


strabismus
4
Work up of the
strabismus patient

Design of the surgical


procedure
Certain principles have proven useful to me in nique. It provides rapid measurement of the refrac-
the surgical management of strabismus over the past tive error after cycloplegia with values similar to
forty plus years. They will be presented here in the retinoscopy carried out by an experienced examiner.
form of guidelines. These guidelines are at times spe- However, the surgeon in training should learn the
cific and at other times general, but in all cases the technique of accurate, on axis retinoscopy. This is the
intent is to present enlightened opinions based on only technique for refraction suitable for the infant,
what I have learned, rather than dogma stubbornly toddler, and preschool child.
held to because it was what I was taught. These
guidelines, when properly applied to a specific case, Step 2
are intended to lead to the design of a surgical proce- Second, the surgeon should be aware of surgical
dure that will be customized to the individual and his options available for a given condition. The surgeon
or her strabismus problem. This plan for surgery should have the ability to carry out these procedures
must be arrived at dynamically with three basic com- with skill and confidence. The importance of tech-
ponents contributing to the ultimate surgical design. nique in performing strabismus surgery cannot be
These components should be determined accurately, overemphasized. The surgeon also must know
understood thoroughly, and combined logically. approximately how much change in ocular alignment
When these logical but simple steps are carried out, will be produced with each procedure in his or her
the answers to the questions of design - which muscle own hands.
and how much surgery - should be clear or at least
clearer to the surgeon. After a plan has been estab- Step 3
lished two additional steps in the surgical manage-
The third step in the design of the surgical proce-
ment of strabismus, surgical technique and follow-up,
dure joins steps 1 and 2 and is the art of strabismus
will be covered.
surgery. This aspect of the surgical design deals with
Step 1 how various types of patients and various categories
of deviations respond to given amounts of surgery.
The ophthalmologist first obtains and records a The anticipated response in a given patient, therefore,
pertinent history and compiles is an accurate, com- modifies the results to be expected from a particular
plete set of measurements that include an accurate procedure. In addition, the most desirable end result
cycloplegic refraction. This latter, often underrated, that could be obtained from surgery such as slight
component of the strabismus evaluation is especially overcorrection or undercorrection can be determined,
important if any hyperopia is present in a patient with but only after careful evaluation has led to a thorough
an esodeviation. If any question remains regarding understanding of the patient. For example, a patient
the accuracy of the cycloplegic refraction, it should with a large deviation will obtain more correction per
be repeated until accuracy is assured. Objective, millimeter of surgery than a patient who has a small-
automated refraction is a useful examination tech- er deviation; esotropic patients with amblyopia may

79
Chapter 4

be overcorrected with the same amount of surgery 2. We frequently use Fresnel prisms in our clin-
that would produce an undercorrection in a nonam- ic for patients who have fusion potential and
blyopic patient; patients with fusion potential should are bothered by diplopia from small-angle
be slightly overcorrected; patients without fusion strabismus which may be changing. These
potential should be undercorrected, and so on. Adults prisms reduce visual acuity proportional to
without fusion potential always look better with a their strength at a rate of approximately 1 line
small-angle exotropia compared to a small-angle per 10 prism diopters. In addition, they can
esotropia.* This extra bit of insight as to how a par- yellow with age, particularly when worn by a
ticular type of patient will respond to a given amount patient who smokes. In spite of these draw-
of surgery helps the surgeon combine each patient's backs, Fresnel prisms continue to be used
needs with the type and amount of surgery required. effectively to relieve diplopia, particularly in
In this way the likelihood of a good result is maxi- the patients with residual strabismus and
mized. especially in the early postoperative period.
It should be understood that all appropriate non- They can also be placed obliquely to treat
surgical treatment that would eliminate the need for small angle horizontal and vertical strabismus
surgery or enhance the results obtained from surgery that coexist. Permanent prisms are used in
including treatment for amblyopia should be carried small-angle residual comitant strabismus
out. These include such measures as the correction of with diplopia in patients when prism therapy
hyperopia in esotropic patients suspected of having is preferred to (repeat) surgery. When cared
an accommodative element to their esotropia and, for properly Fresnel prisms can be worn
when suitable, the use of prisms, anticholinesterase effectively for long periods of time.
drops, and appropriate orthoptic exercises. Botox 3. In some cases of accommodative spasm,
treatment must be considered in selected patients, but chronic atropinization is used to eliminate
in my experience this treatment is indicated in only accommodative convergence and is com-
3% of cases. A detailed discussion of nonsurgical bined with bifocals to treat the near blur. This
treatment of strabismus will not be provided in this temporary treatment is designed to break the
book. cycle of accommodative spasm.
The following specific nonsurgical procedures 4. Occluding contact lenses or high plus contact
are worth mentioning briefly: lenses can be used in one eye in cases of
1. Anticholinesterase drugs are used for treat- intractable diplopia.
ment of refractive-accommodative esotropia. 5. A simple patch may be the only way to
Echothiophate iodide (Phospholine) 0.125% relieve a patients symptoms from diplopia.
or 0.06% used as often as one drop once each Acquired third nerve palsy is the most com-
day in each eye or as little as once per week mon and troublesome cause of intractable
in each eye can reduce or, in some cases, diplopia for adults in our practice.
eliminate esotropia. I use this treatment pri- 6. Pyridostigmine (Mestinon) for the treatment
marily as a diagnostic tool. With moderate- of diplopia from ocular myasthenia gravis is
to small-angle residual deviations in a patient commonly mentioned. However, we have
with fusion potential, anticholinesterase treat- not found that ocular myasthenia responds
ment can be used therapeutically but never well to anticholinesterase treatment.
for an indefinite period. The prolonged use Prednisone by mouth in doses from 10 to 50
of echothiophate iodide can produce iris mg or more every other day in pulsed therapy
cysts. The concurrent use of phenylephrine during episodes of diplopia may be the only
hydrochloride (Neo-Synephrine) 2.5% drops way to maintain comfortable single binocular
once each day reduces the likelihood that vision in a patient with diplopia from the
these cysts will develop. When we use anti- effects of ocular myasthenia.
cholinesterase treatment it is usually for a 7. Therapeutic orthoptics include measures such
specific, predetermined period of weeks or as diplopia awareness, fusional range
months. A common use for anti- enhancement, near point of convergence
cholinesterase treatment is in lieu of glasses training, and supervision of amblyopia treat-
when a child removes them for swimming or ment. The last two are the most commonly
while engaged in similar activities. Another used of the orthoptic treatment modalities.
effective anticholinesterase is disopropyl flu- 8. Therapeutic occlusion for amblyopia is the
orophosphate (Fluropryl) which is supplied in most commonly used nonsurgical adjunctive
ointment form. strabismus therapy. Atropine drops are also

* Look at large paintings of faces on an advertising billboard. The eyes, in order to look aligned, are painted with a large positive angle
kappa making them slightly exotropic. You will also see this in post-Renaissance portraits. Earlier artwork depicts subjects who appear
esotropic or with a reduced pupillary distance.

80
Workup of the strabismus patient

used in one eye to treat amblyopia by creating obtain help if necessary. This reassurance alone will
a blur. allay the patients concerns in most cases. It has been
9. The prism adaptation test is a nonsurgical our practice to include 6 months to 1 year of follow-
technique that helps to predict the response to up in the surgical fee. This makes follow up easier on
surgery in a patient with residual esotropia. the patient and the surgeon! Based on each patient's
To do this test, fully correcting Fresnel prisms situation, we attempt to schedule follow-up without
are placed on the glasses of a strabismus causing financial or other hardship and we individu-
patient with residual refractive esotropia who alize follow-up according to special patient needs.
is also wearing full hyperopic correction. A successfully treated congenital esotropia
Two responses are possible. In one the angle patient who also develops postoperative amblyopia
remains fully corrected with prisms after pro- requires careful follow up to monitor the amblyopia
longed wear and the patient either does or and supervise patching. If the eyes are successfully
does not demonstrate fusion with the Worth straightened, the marker for amblyopia - strabismus -
four-light. These patients have surgery for is gone, making it difficult for the family to detect
the angle measured which is equal to the persistence or recurrence of amblyopia. It is the sur-
amount of prism used. In the other response, geon's responsibility to carry out the visual examina-
the angle of deviation increases so that it tion and supervise the appropriate amblyopia treat-
measures essentially the same or close to it ment in these cases. The incidence of amblyopia in
while wearing the prisms compared to before untreated congenital esotropia is 6% according to
the prisms were placed after an adaptation Calcutt and Murray compared to between 30% and
period of hours to weeks. This response is 50% in large series of operated congenital esotropia
termed eating up the prism or anomalous patients. These data underscore the strabismus sur-
motor response. In this case, additional geon's responsibility for close postoperative follow-
prisms are placed until there is no more up of visual acuity.
response or when up to 60 are placed.
Surgery in this case is performed for the max- Step 1: Patient evaluation
imum angle found; that is, prism plus meas- The initial workup may be recorded on a
ured angle. In cases where the prisms correct preprinted sheet similar to the one illustrated in
the deviation fully in the distance but an Figure 1. The following questions should be
esodeviation remains at near, surgery is done answered and the indicated tests performed and
for the near deviation. This technique was recorded during the process of patient evaluation
subjected to a national collaborative study before strabismus surgery.
which demonstrated a small but statistically
significant improvement in results using History:
information from the prism adaptation test. Why was the patient brought in (why did he or
she come in) for an examination?
Step 4 What have the parents (what has the patient)
The fourth factor in the treatment of strabismus noted about the eyes? ET, XT, hyper, con-
is the surgical technique itself. The care and skill stant, intermittent, closes one eye, tilts head,
with which surgery should be performed will be elevates or depresses the chin, eyes jiggle,
referred to throughout the pages of this book. etc.?
Age of onset - when did the problem begin?
Step 5 Current age
The fifth component of the surgery experience is Birth weight (premature?)
follow-up. The patient's response to surgery should Growth and development
be monitored carefully in the postoperative period. o Present weight
Patching therapy for amblyopia may need to be initi- o Sat up when?*
ated or resumed, anticholinesterase drops may be o Walked when?*
* of interest primarily in infants and young
required, or prisms may be needed. In addition, com-
children
plications of surgery are always a possibility and, if
Any allergies, what medications are currently
present, should be diagnosed and treated promptly. If
being used
there is a complication associated with the surgery or
Significant illness and surgical history
if the result is less than expected - disappointing, you,
the surgeon should acknowledge this. Tell the patient
that you recognize this and be positive, but have
empathy. Let the patient know that you will see the
problem through to either remedy the problem or

81
Chapter 4

Figure 1
A preprinted examination data collection sheet is a useful adjunct to the examination, particularly for the patients first visit.
This form has been effective in our clinic, but in most instances a practitioner will design a form to meet his or her unique
needs. The importance of showing this form is that it includes most of the tests required for a complete examination.
Results of other tests such as passive duction evaluation, tensilon test, exophthalmometry, etc. may be noted on\ this form
in the appropriate area with explanatory labels.

82
Workup of the strabismus patient

Subjective complaints: decimal system this is recorded as 1.0. The letter E


Diplopia (binocular - monocular) with its 5 combined spaces and bars is an ideal target.
Oscillopsia If the smallest object an individual can see at 20 feet
Asthenopia subtends 5 minutes of arc at 200 feet the object is 10
Getting worse or better times larger. This is calculated as 20/200, 6/60, or
Cosmetic issues 0.1. Visual acuity recorded as the viewing distance
Image tilt over the distance the smallest object seen subtends 5
minutes and is recorded: 20/60, 6/18, 0.3, etc.
Occlusion: With an infant or a child too young to cooperate
Which eye for visual acuity testing, reaction to the examiner's
How long face, a light, a non-illuminated (silent) interesting or
How well compelling object, social situations, and, if poor
vision is suspected, to an optokinetic tape or drum
Orthoptics: should be observed. Notation is made describing the
Type of exercises best acuity observed; for example, appears to be (not
How long to be) visually alert to: (a note is made describing the
How well size of the smallest object seen). Strong preference
for one eye associated with objection to occlusion of
Family history: this eye usually indicates amblyopia or an organic
Strabismus - parents, siblings, others visual defect in the other eye. However, even normal
Glaucoma infants tend to object vigorously to occlusion of either
Diabetes eye. Cross fixation, or using the eyes alternately,
Other eye problems rules out amblyopia. Dim illumination is best for
Trouble with anesthesia (malignant hyper- evaluation of vision in the neonate or very young
thermia) infant. Simply turning off the overhead lights may
turn a recalcitrant newborn with closed lids into an
General health: interested patient able to fixate on the examiner's
Trauma history face.
Diabetes The preferential looking technique using Teller
Fatigability acuity cards has been used to quantify resolution acu-
Developmental delays ity in the newborn and infant. This method can be
employed to determine an objective value for visual
Special characteristics: acuity in a very young child. However, to determine
Head tilt (nodding) relative acuity in order to establish the presence of
Preferred eye preference of one eye for fixation and therefore
Variability of deviation amblyopia, simple clinical observation of fixation
Face turn preference is sufficient to detect a difference in visu-
Dancing eyes (nystagmus) al acuity of one octave in most young patients.
Facial asymmetry An E chart or a STYCAR (HOTV) chart visual
acuity test can usually be accomplished at the earliest
Prior treatment: with girls at age 3 and with boys at age 3 1/2,
Glasses although exceptions do occur. Linear testing with E's
o When prescribed or letters is valid. Testing with isolated E's gives erro-
o Prescription neously good vision results in the presence of func-
Bifocals tional amblyopia because of the absence of the
Prisms crowding phenomenon. Crowding can be achieved
resulting in accurate vision testing even with isolated
Ocular surgery: optotypes by using crowding bars. These are lines
When equal to the width of the optotype segments placed at
What was done each side and above and below the single optotype
By whom used for visual acuity testing. We use isolated E's
mainly for instruction. Vision in older children is
Visual acuity testing determined with the letter chart. In children where
Visual acuity is recorded as the smallest object a testing with the E chart cannot be accomplished, Lea
patient can see at a given distance. 20/20 vision (or symbols or other recognizable pictures can be used.
6/6 vision) means that at 20 feet (approximately 6 The Lea symbols are particularly useful to demon-
meters) a patient can see an object that subtends 5 strate a difference in visual acuity between the two
minutes of arc with components of 1 minute. In the eyes in a child too young to cooperate for other visu-

83
Chapter 4

al acuity testing. In an office or clinic setting, com- acuity test or with the Worth four-lights. In this case,
puter generated visual acuity testing provides all of the word fusion is recorded. In the presence of stra-
the visual acuity testing options while at the same bismus, if one eye is preferred for fixation and the
time providing standardized illumination, random other eye deviates, the notation is fixation OD or fix-
display, and more. This instrumentation is useful but ation OS. If either eye is used for fixation with free
expensive. Relative visual acuity is alternation between the eyes or cross fixation is noted
more useful clinically than absolute acuity in an (the left eye fixes in right gaze and the right eye fixes
infant suspected of having amblyopia. Recognition in left gaze) free alternation may be present and is
acuity with Snellen optotypes (E's, the alphabet, or recorded as such. A patient may prefer one eye but
symbols) is a more stringent and, therefore, more hold fixation briefly with the non-preferred eye after
accurate test than resolution acuity done with stripes. the cover has been removed from the preferred eye.
For this reason, we test vision with Snellen optotypes Such a patient usually does not hold fixation in the
determining recognition acuity whenever we can non-preferred eye through a blink. This type of acu-
obtain cooperation from a child. ity response is recorded as prefers OD, will hold but
If decreased vision is found in each eye when not take up fixation OS, will not hold fixation through
checked monocularly, vision should be checked a blink, etc.
binocularly. The examiner in this case should be Gross, wandering fixation may be present in the
looking for latent gross or micronystagmus. Fogging non-preferred eye and should be recorded as such.
with a plus lens may be used to block vision in one Nystagmus, if present, is noted and characterized as
eye but avoid nystagmus when determining monocu- latent or manifest according to when the nystagmus
lar visual acuity in a patient with latent nystagmus. is present, and horizontal, rotary, vertical, pendular,
Near vision should be checked with E's, isolated let- jerk, and the like according to the pattern of nystag-
ters or numbers, or sentence reading, depending on mus. Frequency, amplitude, positions of greater
age. intensity, and null point are also described.
Visual acuity testing with neutral density filters Nystagmoid movements differ from nystagmus in
can differentiate functional from organic amblyopia. that the former are non-rhythmic and usually result
Vision in an eye with functional amblyopia remains at from a sensory rather than a motor defect.
or near the same level when neutral filters of increas- A peculiar type of ocular motility and head pos-
ing density are introduced. Vision in a normal eye or ture anomaly occurs with ocular motor apraxia. With
in an eye with an organic cause for poor vision this condition the eyes do not move in response to
decreases proportionally with the increased density of voluntary attempts at binocular vision through ver-
the filter. sions. Instead, the head moves past the point of fixa-
Near point of accommodation is determined in tion on the object of regard bringing the eyes along,
cooperative patients by moving a card with small so to speak. Then when the eyes have established fix-
print closer to the patient until the blur point is ation on the intended object, the head rotates back
reached. The near point of accommodation is record- while the eyes maintain fixation on the object. This
ed in diopters or centimeters. enigmatic supranuclear congenital condition tends to
Pupillary response to light is evaluated by mov- improve with age and requires no specific treatment.
ing a light from in front of one eye to in front of the Asymmetric, often unilateral, horizontal nystag-
other in reduced ambient light. A Marcus Gunn affer- mus with head nodding and torticollis in an otherwise
ent pupillary defect indicating decreased optic nerve normal child approximately 1 or 2 years old may be
function can be demonstrated with this test by noting spasmus nutans. This is a benign condition that also
a dilated pupil with the light shining in the involved requires no treatment. Spasmus nutans always
eye (direct response) and constriction of the pupil improves. Unless this condition can be clearly differ-
when the light shines in the fellow eye (consensual entiated from potentially serious causes of acquired
response). With the swinging flashlight afferent nystagmus such as chiasmal glioma or posterior fossa
pupillary defect test, as the light moves rapidly back tumor, computed tomography (CT) or magnetic reso-
and forth, both eyes dilate when the light is shining in nance imaging (MRI) of the head should be per-
the abnormal eye and both eyes constrict when the formed. There are widely differing points of view
light is shining in normal eye. regarding imaging in cases of apparent spasmus
nutans. Some pediatric ophthalmologists and neuro-
Preliminary evaluation of binocular ophthalmologists advocate imaging in every case
function while others, including myself, will do imaging only
in cases where an additional physical sign is present
Fixation such as fussiness, weight loss, or some other added
If neither eye is preferred for fixation and if the sign.
eyes appear grossly straight, fusion may be present or Vertical nystagmus with retraction is a sign of a
at least apparent. This can be confirmed with a stereo lesion around the chiasm. Patients with retraction

84
Workup of the strabismus patient

nystagmus should be studied with appropriate imag- Sensory evaluation


ing. Any vertical nystagmus, particularly when
acquired, should be evaluated with a neurologic In selected cases, certain sensory tests should be
workup including CT or MRI. Downbeating nystag- performed after the history has been taken but before
mus may be associated with Arnold-Chiari malforma- the eyes have been dissociated with covering and
tion and upbeating nystagmus with rostral brain stem before visual acuity has been determined. Some
lesions. Rapid, flutter movements are associated with patients are able to fuse in casual seeing situations
neuroblastoma. Acquired nystagmus in childhood is that are not stressful, but lose their weak hold on
always a matter of concern. It is the responsibility of fusion after the slightest dissociation. Patients with
the examining physician to make a decision about any type of intermittent deviation and bifoveal or
further workup and/or referral and at the very least to peripheral fusion should have their stereo acuity
maintain careful follow up, such as three month inter- determined initially and then should be tested with
vals. the Worth four-dot test before resuming the more
usual flow of the examination. In addition, stereo
Ductions acuity testing is a good screening device for patients
Ductions or monocular movements are evaluated in whom the surgeon suspects the examination will be
in extreme abduction, adduction, sursumduction, and normal. Any patient who accurately sees nine out of
deorsumduction. In cases where abduction is avoid- nine Titmus vectographic targets and, therefore, can
ed such as occurs in congenital esotropia with cross appreciate 40 seconds of arc disparity, is unlikely to
fixation where the right eye looks left and the left eye have a significant problem with their binocular mech-
looks right, ductions are tested with the Dolls head anism or visual acuity.
test (Figure 2). This is also called the oculocephalic
Haploscope examination
reflex test. This test is done by rotating the head rap-
idly to one side while particularly observing the eye This testing requires an instrument which stimu-
opposite the direction of head rotation. Ductions are lates the eyes separately but provides images with
graded 1+ to 4+ overaction (this is a relative value) varying degrees of similarity and is designed to test
and -1 to -4 underaction. Forced ductions, muscle- the ability of the eyes to work together in response to
force generation, and saccadic speeds are determined a variety of stimuli. This testing is ordinarily done by
in patients with significant limitation of ductions. an orthoptist.

Head posture
Any face turn, chin elevation or depression, or
head tilt is noted and recorded. This observation is an
especially helpful clue in patients with vertical mus-
cle palsies and strabismus with limitation of ductions
where fusion is present. An anomalous head posture
is also a prominent feature of nystagmus with null
point. A bizarre head posture may be assumed to aid
in fusion or even in some cases to increase the amount
of diplopia to aid suppression. A simple diagram
showing the head posture and direction of gaze can be
useful. At this point any facial asymmetry, a common
finding in congenital superior oblique palsy, clefting,
skin tags, hypertelorism, unusual palpebral fissure
characteristics, etc. should be noted.
Screen comitance
Versions, or binocular eye movements, are eval-
uated in the extreme diagnostic positions comparing Figure 2
movements in the extremes of gaze carried out by Dolls head, oculocephalic response to elicit lateroversions
yoked muscles. Arrows and hash marks on the record (abduction), in an infant.
indicate overaction or underaction of the muscles.
Arrows outside the figure indicate overaction and
hash marks on the lines denote underaction. The lines
drawn as paired H figures represent the field of action
rather than the location of the yoked extraocular mus-
cles.

85
Chapter 4

First-degree fusion
The objective angle is determined after dissocia- refused at convergence of 28D. The presence of sec-
tion with the haploscope. It represents the patient's ond-degree fusion indicates that a functional result
manifest or latent strabismus (total fusion free devia- with fusion and fusional amplitudes should be obtain-
tion). Since the two targets are presented alternately, able with proper surgery. Such patients even when
this is essentially the same angle found with alternate slightly overcorrected by surgery are those best able
prism and cover testing. The subjective angle is the to obtain excellent long-term results. Fusional ampli-
angle at which the patient superimposes images of tudes can be measured in free space using the princi-
objects by manipulating the arms of the amblyoscope. ples of the haploscope but shifting images seen by the
These angles are determined clinically using dissimi- two eyes with a prism bar containing horizontal
lar, incomplete, grade I, simultaneous macular per- prisms of gradually increasing strength from 1 to
ception slides in the arms of the major amblyoscope 40D.
or other haploscopic devices; for example, a lion in a Stereo acuity. This has been called third-degree
cage, etc. Comparison of these two angles indicates fusion, but it should be recognized that these degrees
the status of retinal correspondence, at least at the of fusion are not a continuum but actually test differ-
level of dissociation created by the amblyoscope. ent things: first degree - retinal correspondence; sec-
When the objective and subjective angles are the ond degree - motor fusion; third degree - sensory
same, retinal correspondence is normal. When the fusion. The Titmus vectograph is used to test stereo
subjective angle is zero and the objective angle is acuity. Findings with this test are recorded as fly
either plus (base out esodeviation) or minus (base in (3000 seconds arc disparity); A, B, C animals; and the
exodeviation), harmonious anomalous retinal corre- fraction of the nine dots that the patient can appreci-
spondence is present. When the subjective angle is ate. The ninth dot on the Titmus vectograph describes
less than the objective angle but other than zero, non- 40 seconds of arc disparity.*
harmonious anomalous retinal correspondence is Stereopsis is not recordable in manifest strabis-
present. If no subjective angle can be determined mus of sufficient size to warrant surgery. However, it
with grade I slides, first-degree fusion is absent. may be quite good in intermittent deviations such as
First-degree fusion and normal retinal correspon- intermittent exotropia even with large angles or in
dence are favorable but by no means certain indica- small angle manifest strabismus with peripheral
tions that a functional result with fusion may be fusion.
obtained from surgery. Stereo acuity is tested using the polarized vecto-
Second-degree fusion graph method that measures the ability to fuse later-
ally displaced objects within Panums fusional space
Range of fusion. If a subjective angle is found
producing the illusion of depth. But a drawback of
with appropriate slides, grade II fusion targets are
this test is that careful study of these images can offer
inserted into the arms of the major amblyoscope and
monocular clues. Random dot vectographs have
the patient's fusional amplitudes are determined.
embedded disparity not seen monocularly. This so-
Grade II fusion slides are similar in their overall out-
called global stereopsis is said to provide more accu-
line and differ only in detail. These differences serve
rate findings of stereo acuity. Both of these tests
as checkpoints ensuring that both eyes are seeing a
require viewing with polarized glasses. Stereo acuity
target. With grade II targets in the amblyoscope, the
can also be measured without the use of spectacles
arms are first shifted from the subjective angle out-
using the Lang test or the Frisbie test. Stereo acuity
ward (exo) and then inward (eso). Fusional ampli-
can also be measured in free space using the Howard-
tudes are an expression of the patient's ability to keep
Dolman apparatus which requires the examinee to
the images as one and, therefore, fused by either
align distant objects. This test is used primarily in
diverging or converging the eyes as the arms of the
clinical research settings.
amblyoscope are shifted outward and inward.
Worth four-dot testing. Worth four-dot testing is
Fusional divergence is usually tested before fusional
performed at variable near distances and at 20 feet.
convergence. A make and break point for each is
Results of this testing are recorded as fusion, diplop-
recorded; for example, -6 to -4 and +40 to +28. This
ia, alternation, or suppression of one eye. In many
means that the patient experienced diplopia when the
instances, patients with small-angle esotropia, central
arms got to 6D exodeviation but was able to refuse
suppression, and peripheral fusion will fuse a four-
the images as the arms were returned to 4D exodevi-
light pattern that produces a large retinal image but
ation; fusion was held to 40D of convergence before
will suppress one set of lights when the retinal image
diplopia appeared and the doubled images were
is made smaller either by reducing the size of the tar-

*Titmus vectograph findings in seconds of arc disparity are: fly = 3000, cat = 400, rabbit = 200, monkey = 100, 1/9 = 800, 2/9 = 400, 3/9
= 200, 4/9 = 140, 5/9 = 100, 6/9 = 80, 7/9 = 60, 8/9 = 50, 9/9 = 40.

86
Workup of the strabismus patient

get or increasing the viewing distance. A gross esti- the lines do not intersect at the fixation point, deep
mation of the size of the central functional scotoma anomalous correspondence is present.
present during binocular vision in patients with stra- Sensory fixation. Fixation behavior is deter-
bismus can be made by determining how far the four mined with an ophthalmoscope that contains a fixa-
lights must be removed from the patient and, there- tion target that the patient is asked to look at with
fore, how small the retinal image becomes before each eye while the fellow eye is occluded. The exam-
suppression occurs. The size of the retinal image cre- iner can compare the retinal point used to fixate this
ated by the four lights as the lights recede from the target with the anatomic location of the fovea. This
patient can be calculated, but the precise value is not point of fixation is recorded directly on the chart with
clinically important. The Worth four-dot test may a small x. Fixation with any retinal point other than
also be considered a gross color vision test and a test the fovea means that acuity in that eye will be
of retinal correspondence. If four lights in proper reduced. The farther the point of fixation is from the
alignment are seen in the presence of a manifest stra- fovea, the greater the reduction in acuity. Amblyopia
bismus, harmonious anomalous retinal correspon- with peripheral eccentric fixation suggests the
dence may be inferred. This is a gross test and not possibility of a significant overcorrection of an
one that is likely to alter decisions regarding treat- esodeviation even when moderate surgery is done.
ment, although four light fusion is considered a favor- Implications of sensory testing. Sensory testing
able finding with the prism adaptation test. is useful both preoperatively and postoperatively.
Bagolini striated glasses. Bagolini glasses are The closer to normal the preoperative sensory find-
essentially see-through, micro Maddox rods that ings, the more the surgeon should try to create surgi-
turn a point of light into a line while not disturbing cal alignment or a slight surgical overcorrection that
vision. These glasses are ordinarily placed in a trial would lead to fusion. Postoperative sensory testing is
frame with their axes at 135 OD and 45 OS (or a check on surgical results and a guide to further non-
equivalent). They are used to determine retinal cor- surgical treatment that should be pursued appropriate-
respondence in casual seeing. Nearly all strabismic ly in the case of an undercorrection or an overcorrec-
patients when viewing a point of light will see diago- tion in a potentially fusing patient.
nal lines intersecting at the light or where the light is
stimulating the retina in cases with a small central Measurement of alignment - prism
scotoma. This finding is compatible with harmonious cover testing and other methods
anomalous retinal correspondence in the strabismic
Alternate prism and cover testing is a method to
patient. Some patients will see one diagonal line cor-
measure the maximum deviation. This testing is per-
responding to the preferred eye while suppressing the
formed at distance (20 feet) and near (13 inches), with
non-preferred eye. Other patients will see a complete
and without glasses (if they are worn) while the
line corresponding to the preferred eye and an incom-
patient views an accommodative target in the primary
plete line, with a missing segment adjacent to the
position. The use of an accommodative target and the
light, with the non-preferred eye. These test results
wearing of glasses are essential because together they
ordinarily do not influence surgical planning. These
control the patient's accommodative convergence.
have been used by investigators for a variety of diag-
Prism and cover testing is also done in approximate-
nostic and therapeutic manipulations and for the
ly 30 of upgaze and downgaze while the patient
study of retinal correspondence and abnormal binoc-
wears full correction and views an accommodative
ular movement.
target in the distance. If this test is performed at near
After-image test. The afterimage test is used to
while measuring for A and V pattern, the patient
determine retinal correspondence in extreme dissoci-
should wear +3.00 D lenses over the distance correc-
ation. Anomalous retinal correspondence occurring
tion to eliminate the effects of accommodative con-
on the afterimage test indicates a deep sensory anom-
vergence, especially in downgaze.
aly. Retinal correspondence tends to be normal or
Upgaze and downgaze can be achieved by tilting
harmonious anomalous when tested with the Bagolini
the patient's head forward and backward. This
glasses, normal or anomalous with the haploscope,
maneuver uncovers an A or V pattern which is best
and normal with the after-image test, indicating that
tested while the patient fixes on a distant target. A
retinal correspondence response varies with the test-
10 difference between upgaze and downgaze is sig-
ing conditions and the test. All that can be inferred
nificant for diagnosing an A pattern and a 15 differ-
from this testing is how completely the eyes have
ence is significant for a V pattern.
adapted to the strabismus angle. To do an afterimage
Other useful variations of prism and cover test-
test, a bar of bright light with a non-illuminated cen-
ing that can be performed before or after the alternate
tral fixation point is presented horizontally to the eye
prism and cover test include the following:
used for fixation and then vertically to the other eye.
1. The cover-uncover test differentiates a
If the afterimage intersects at the fixation point, nor-
tropia from a phoria. Both are measured at the
mal retinal correspondence is present with this test. If

87
Chapter 4

same time but not differentiated with the light reflex in the non-fixing eye, approxi-
alternate prism and cover test. Movement of mately 7 degrees or 15 of deviation is pres-
the covered eye immediately after the cover ent.
is removed and while the uncovered eye 6. The Krimsky test determines the amount of
maintains fixation indicates a phoria. A prism that must be placed before the fixing
tropia is noted by first establishing the fixing eye to center the corneal light reflex in the
eye and then covering it while observing the pupil of the non-fixing eye as this eye pas-
fellow eye for movement. If the fellow eye sively moves according to Herings law (see
does not move, the patient is orthotropic. If page 105). This test is particularly useful
the eye moves to take up fixation, a tropia is when the patient has such poor vision in one
present and the direction should be noted. If eye that fixation is not taken up well with that
the eyes move inward toward the nose, an eye during prism and cover testing.
exoshift is recorded; if the eyes move outward 7. Prism and cover testing with either eye fix-
toward the ear, an esoshift is recorded. When ing in the nine diagnostic positions of gaze
the eyes appear straight and/or good stereo is performed in cases of muscle palsy, partic-
acuity has been measured, even while assum- ularly vertical muscle palsy. This is the pre-
ing a face turn, head tilt, etc., the surgeon mier measurement of alignment.
should proceed to the motor evaluation. The 8. Alternate prism and cover testing with the
patient could have an incomitant mechanical head tilted approximately 45 to the right
strabismus dealt with by the patient with a and to the left is called the Bielschowsky test.
face turn, head tilt, or both. This would have This test which is said to be positive when a
been uncovered for example in a case of vertical deviation increases with head tilt is
Brown or Duane while testing ductions. A useful for identifying isolated cyclovertical
phoria is the most important feature found muscle palsy.
with the cover-uncover test. With aligned 9. Dissociated vertical deviation (DVD) is
eyes in casual seeing, a phoria can be meas- noted when either eye drifts upward the same
ured with alternate prism and cover testing. or differing amounts when occluded and
This testing also measures the total deviation, down when the cover is removed with cover-
phoria, and tropia when these coexist. uncover testing. It is recorded as +1 (5) to
2. Lateral gaze prism and cover testing can +4 (25). Some surgeons prefer to measure
reveal the presence of lateral incomitance rather than estimate DVD. This measure-
which is especially important in exodevia- ment may be performed in a manner similar
tions and in previously operated patients. to that with the simultaneous prism and cover
3. Prism and cover testing with either eye fix- test. However, DVD may be present with a
ing helps to determine the primary and sec- coexisting true vertical deviation that is in the
ondary deviation. This test is a variation of same or opposite direction as the DVD.
the simultaneous prism and cover test. DVD is also often of different amplitude in
4. Simultaneous prism and cover testing various fields of gaze and may demonstrate
(SPC) determines the actual tropia in casual movement in only one eye! DVD may even
seeing in patients where a tropia and phoria present as pseudo-overaction of the inferior
coexist (monofixational esophoria, monofix- obliques. This is confirmed by noting a
ation syndrome, microstrabismus, or small- hyperdeviation of the occluded, abducted eye
angle tropia with peripheral fusion). The fix- during lateral gaze. With true inferior
ing eye is first identified. Then it is covered oblique overaction, and no DVD the occlud-
while a prism of appropriate size and orienta- ed abducted eye is more likely to be hypode-
tion is simultaneously placed in front of the viated. Also, a V pattern should be present
deviating eye. The amount of prism is with true inferior oblique overaction. An eye
increased or decreased until no movement with DVD that moves upward when covered
occurs in the deviating eye. The prism need- can drift well below the midline when the
ed to preempt re-fixation with the deviating cover is removed. This phenomenon has
eye is a measure of the alignment during been called a falling eye. Some eyes with
casual seeing. very poor vision will drift below the midline
5. The Hirschberg test compares the location with an accompanying vertical bobbing of
of the light reflex which is normally in the the eye. This is called the Heiman-
center of the pupil of each eye to the anatom- Bielschowsky phenomenon. An exodevia-
ic central pupillary axes. It is performed tion of one eye only is called dissociated hor-
when patient cooperation is poor. For each izontal deviation (DHD). This is in a way an
millimeter of displacement of the corneal extension of DVD. The two have been char-

88
Workup of the strabismus patient

acterized as the dissociated strabismus com- afternoon. Use of atropine is usually restricted to pre-
plex (DSC) by M. E. Wilson. school-aged children, for initial refraction, and when
10. A translucent occluder* held before one eye esotropia is present.
forces fixation with the other eye but allows When refracting a lightly pigmented patient in
observation of the occluded eye. This is an the usual office setting, a cycloplegic refraction can
excellent way to observe the deviation in be performed satisfactorily approximately 20 to 40
DVD. This testing is used effectively in tele- minutes after one or two drops of cyclopentolate
medicine (see chapter 15). (Cyclogyl) 1% have been instilled in the cul-de-sac in
11. Red lens and Maddox rod tests are useful children over 1 year. One drop of phenylephrine
subjective tests and for charting in cases of (Neo-Synephrine) 2.5% may be used in addition to
small-angle vertical and/or horizontal strabis- the cyclopentolate in patients with dark irides. In
mus with symptomatic diplopia. children under 1 year of age, cyclopentolate 0.5%
12. The double Maddox rod test is useful in the drops are used.
diagnosis and measurement of cyclodevia- In esodeviating patients the full hyperopia must
tions. be elicited. Hyperopia as low as +3.00 D should
13. The 4 base-out prism test may be used to receive a trial treatment with glasses in patients with
uncover a scotoma in the macula of one eye esotropia usually beginning after 1 year of age.
in patients with microtropia. However, I have seen several patients less than 1 year
14. A Hess, Lancaster, or Lees screen may be of age with esotropia relieved by wearing a +3.00 D
used to plot directly the deviation in a coop- correction. Echothiophate iodide (Phospholine)
erative strabismic patient. drops (0.06% to 0.125%, one drop in each eye each
15. Diplopia fields are mapped with a Goldmann morning for 3 weeks) in lieu of glasses may help to
perimeter or arc perimeter while the patient determine what effect the hyperopia (accommodative
views the moving fixation target with both effort) has on the esodeviation, but because anti-
eyes open and with the head centrally posi- cholinesterase drops only reduce the effective accom-
tioned and stabilized. The documentation modative convergence/accommodation ratio (AC/A)
provided is valuable for following recovery and do not eliminate the need for accommodation,
from an acute paresis and especially for they are not a true substitute for glasses.
medicolegal and compensation purposes. A difference in the refractive error between the
eyes is called anisometropia. Difference of as little as
Refraction +1.00 or +2.00 diopters can be amblyopiogenic and
Refraction has a vital role in the diagnosis and usually indicates the need for the full refractive dif-
treatment of strabismus. It is imperative that those ference to be prescribed. If glasses are prescribed
involved in the care of the strabismus patient under- with the hyperopia reduced, it should be reduced
stand the principles of refraction, have the skills to equally. For example, OD +2.00 +1.00 x 90, OS
perform accurate measurement of the refractive error, +6.00 +2.00 x 90, could be cut to OD +1.00 +1.00 x
and use spectacle correction or the equivalent as 90, OS +5.00 +2.00 x90. These glasses would be
required in the treatment of strabismus. If you intend effective and better tolerated.
to treat patients with strabismus but are not compe- Adult patients do not ordinarily require cyclople-
tent with refraction technique, you should stop here. gia, but in selected cases of convergence excess this
Learn how to refract and then resume your study. A may be needed. It is best to avoid use of cycloplegics
person ignorant of refraction methods is not fully in pre-presbyopic adults. Use of cycloplegics in such
qualified to treat strabismus! patients can precipitate presbyopia resulting in a very
Work up of the patient with strabismus always unhappy patient.
starts with the refraction. In heavily pigmented Fundus examination
patients a cycloplegic refraction is performed using
atropine solution 1.0% (1 drop) or atropine ointment Examination of the retina is carried out using a
1% (-inch strip) in each eye for 3 days before the day standard or small portable indirect ophthalmoscope.
of examination. When drops are used, the atropine It is a relatively simple matter to see the retina poste-
may also be applied on the day of the examination. rior to the equator in a squirming infant using the
Careful instructions are given to the parents to avoid indirect ophthalmoscope. This examination to rule
overdosage. These instructions include using no out pathologic conditions in the posterior pole is an
more medicine than prescribed and holding a finger essential part of the evaluation of every strabismus
over the punctum for 30 seconds after drops are patient. Remember, esotropia is the second most com-
instilled in one eye in the morning and the other in the mon presenting sign, after white pupil of retinoblas-

* This technique was made popular by Annette Spielmann of Nancy, France.

89
Chapter 4

toma! The direct ophthalmoscope is used to study 7. Forced ductions


detail of the optic nerves and maculae and to deter- 8. Generated muscle force
mine fixation behavior in the older child with ambly- The diagnosis should include as much informa-
opia. A total retina examination such as that needed tion as possible about the strabismus problem includ-
to rule out retinoblastoma in an infant requires an ing notation of some or all of the following:
examination under general anesthesia. For purposes 1. Etiology
of strabismus, examination of the posterior retina as it 2. Direction of deviation (eso, exo, hyper,
effects central vision is sufficient. cyclo)
3. Comitance
Biomicroscopic examination 4. Fixation behavior
Examination of the anterior segment should be 5. Vision (amblyopia)
performed on all patients, particularly when echothio- 6. Refraction, accommodative factors
phate iodide is used, because of the possibility of iris 7. AC/A
cysts and to confirm continued clarity of the lens. A 8. Manifest or latent
portable slit lamp is a valuable instrument for exam- 9. Constant or intermittent
ining children under 3 years of age. At the very least, 10. A or V pattern
the anterior segment should be studied using the 11. Mechanical factors (forced ductions)
direct ophthalmoscope. When nystagmus and 12. Cranial nerve status (saccadic velocity -
decreased vision are noted, iris transillumination secondary deviation)
defects indicative of albinism, either oculocutaneous 13. Muscle force
or ocular, should be looked for. 14. Lid fissure configuration
15. DVD - DHD
External examination 16. Head posture
The appearance of the lids should be noted. Is 17. Aberrant regeneration
there ptosis or epicanthus (superioris or inferioris)? 18. Oblique muscle function
Are the palpebral fissures mongoloid, anti-mon- 19. Facial asymmetry
goloid, or normal? Is there proptosis, hypertelorism, 20. Ptosis (upper or lower lid)
microphthalmos? Is lid lag present? Are there con-
junctival scars? All of this information should be
Diagnostic considerations for
recorded. strabismus with restrictions
Diagnosis of the strabismus condition Clinical evaluation of strabismus
When sufficient historical data and motility and with restricted motility
refractive measurements have been recorded, a diag-
Underaction
nosis is made. This diagnosis can be made in most
cases after the initial examination. Repeated meas- Apparent excesses or real deficiencies of ocular
urements at one or more subsequent visits should be movements can occur with longstanding strabismus.
taken in some cases before a final quantitative diag- Even more dramatic and obvious apparent excesses
nosis is established and a specific surgical treatment and real deficiencies occur with acute palsies and
plan determined. For example; congenital esotropia, with congenital restrictive strabismus such as with
longstanding superior oblique palsy, sensory Brown, Duane, Mbius, or fibrosis syndromes. Other
esotropia or exotropia and similar types of strabismus causes of strabismus with restriction include metabol-
can usually be scheduled for surgery at the initial visit ic (thyroid ophthalmopathy), iatrogenic (postopera-
and then re-evaluated on the day of surgery. In con- tive eye muscle surgery, cataract, or retinal detach-
trast, intermittent exotropia in the toddler, acute cra- ment surgery), traumatic (blowout fracture), fibrosis
nial nerve palsies, and residual refractive esotropia syndrome, progressive ophthalmoplegia, orbital
may require a longer period of observation along with tumor, inflammation, and more. Accurate analysis of
repeated measurements. the sometimes subtle abnormalities in the extent and
Other special examinations may be employed character of ocular movement caused by acute or
for complex diagnosis including the following: longstanding strabismus, or from any of the other
1. Tensilon test causes listed, is an essential step in the planning and
2. Differential intraocular pressure test ultimate execution of the correct surgical procedure.
3. Saccadic velocity observed and with elec- For example, in an esotropic patient with deficient
tro-oculogram recording abduction in the chronically esodeviated eye and oth-
4. Nystagmography erwise full unrestricted ductions, the correct surgery
5. Iris angiography would be dictated by the results of testing both active
6. Imaging of the orbit with CT or MRI and passive movement in the deviated eye. In such a

90
Workup of the strabismus patient

patient, any mechanical restriction to abduction must normal eye in cases of superior oblique palsy with
be released before the eye could be straightened and restriction of the ipsilateral superior rectus. This is
before there could be any hope of normal or near nor- due to a Herings law response with the normal supe-
mal abduction. Whether release of the medial restric- rior oblique receiving the same innervation as its
tion alone would be sufficient or whether ipsilateral yoke, the inferior rectus, in the involved eye.
lateral rectus resection or even a muscle transfer Congenital or acquired incomitant strabismus
should be performed would depend on the results of with limitations in motility caused by muscle palsies
testing for generated muscle force. If, for example, or mechanical restrictions, or a combination of the
generated force were brisk and the angle of deviation two, is usually easy to recognize. However, an accu-
small, freeing of the restriction with or without medi- rate and correct diagnosis in such cases is absolutely
al rectus recession could be sufficient. If the angle essential before a plan for surgery can be determined.
were large and the generated force only moderate, lat- Nowhere in strabismus treatment is proper diagnosis
eral rectus resection in addition to the medial rectus more essential to proper execution of surgical skills
recession might be necessary. Finally, if generated than in these cases of incomitant strabismus. The dif-
force toward abduction were minimal or nil, it would ferential diagnosis of strabismus with restricted
be necessary to do some type of extraocular muscle motility requires analysis of saccadic movements,
transfer procedure. Whether the medial rectus were forced (passive) ductions, and muscle force genera-
recessed or weakened with Botox would depend on tion in addition to the usually performed prism and
the degree of restriction and to some extent on the cover tests.
angle of deviation as well as on the surgeon's own Evaluation of noncomitant strabismus
preference.
The example shown in Figure 3 is a model for
Except in certain cases of total sixth or third
any type of strabismus with restricted motility in one
nerve palsy and a few other examples, such as inferi-
or both eyes and in one or more fields of gaze. The
or rectus denervation as occurs in certain cases of
analysis becomes more complicated as more muscles
blowout fracture, some eye movement force remains
and fields of gaze are involved, but the principles are
in the so-called underacting muscle. This remaining
the same. The patient has a right esotropia in the pri-
force, though diminished, points out that emphasis
mary position. This is the primary deviation and
should be placed on freeing of mechanical restric-
measures 50. In levoversion the eyes are grossly
tions. In some cases this is relatively easy, such as in
parallel, with normal adduction of the right eye and
cases of longstanding strabismus with a normally
normal abduction of the left eye. No muscle weak-
contracting antagonist. On the other hand, freeing of
ness or mechanical restriction is apparent in this
the restriction in a congenital Brown syndrome to
direction of gaze. In dextroversion the right esotropia
restore normal or near normal ocular motility can be
increases markedly. Abduction of the right eye is
difficult to accomplish. In addition, freeing of
deficient, whereas adduction of the left eye is normal
restricted movements can result in a distressing over-
or could be increased. The decreased abduction in the
correction.
right eye could be the result of a paretic right lateral
Overaction rectus, a mechanical restriction associated with the
Overaction of extraocular muscles is more diffi- right medial rectus or with various muscular or fascial
cult to analyze and categorize than is underaction of structures in the right eye, or a combination of the
extraocular muscles. For this reason, we use the term two. When the patient fixes with the right, paretic
apparent overaction. There is no evidence that the eye the left esotropia is increased to 70. This is
so-called overacting muscle has more force or has a called the secondary deviation.
greater saccadic velocity. Even though the eye
appears to move farther in the usual field of action. Saccadic velocity analysis
This is seen as excess adduction or abduction in long- Observed saccadic velocity is a useful clinical
standing esotropia or exotropia. This overaction may tool for differentiating a weak muscle from a normal
be seen because the checking of muscle action is muscle, which underacts only because it is held in
relaxed or relatively ineffective. In the case of over- check by mechanical restrictions. A normal extraocu-
acting oblique muscles, the apparent increase in lar muscle will produce a brisk movement of the eye
action may be a horizontal movement which allows with a peak velocity of 400 per second to as high as
the globe to move farther laterally in the orbit. 600 per second. A paretic muscle will usually pro-
Careful observation of the apparent overacting supe- duce velocities at about one-tenth this speed. This
rior and inferior oblique frequently reveals that over- movement may be produced not by the paretic mus-
action is actually abduction. This produces the well- cle but by elastic orbital forces influenced by relax-
known V with overaction of the inferior obliques ation of the antagonist of the paretic muscle.
and A with overaction of the superior obliques. Decreased saccadic velocity can be readily observed
Overaction of the superior oblique can be seen in the in the clinical setting. The electro-oculograph pro-

91
Chapter 4

Figure 3
A Fixing with the sound eye (primary deviation) - a right C Dextroversion shows limitation of abduction in the right
esotropia is observed. eye.
B Levoversion is essentially normal. D Fixing with the paretic eye and/or mechanically restrict-
ed right eye produces a larger secondary deviation.

92
Workup of the strabismus patient

vides recordings useful for determining subtle defi- function is in question. The patient is then asked to
ciencies and for purposes of documentation in the abruptly switch fixation to an object in the field of
laboratory. But for clinical evaluation, observation action of the suspected paretic muscle. In this case
alone is usually sufficient. the patient is asked to switch fixation from extreme
Saccadic velocity analysis begins with the patient levoversion to extreme dextroversion. To accomplish
fixing on an object in the field opposite that of the this maneuver, it is best to instruct the patient to look
suspected paretic muscle (Figure 4). The patient at an object that the examiner holds in the patient's
shown has a right esotropia. To begin the test for sac- far left field and then to look at an object held in the
cadic velocity, the patient is asked to look far to the examiner's hand in the patient's far right field. During
left in the field opposite that of the muscle whose the patient's switch of fixation, the examiner observes

Normal innervation

Paresis v. paralysis

Figure 4
A Eyes are in a gaze opposite the field of action of the C A slow or floating saccade indicates decreased innerva-
underacting muscle tion of the right lateral rectus. No information is avail-
B Fixation shifts from extreme left to extreme right gaze able about possible mechanical restriction.
resulting in a saccade. In this case the eyes move with
equal velocity but excursion of the right eye is limited
suggesting normal innervation to the right lateral rectus.
Restricted right eye movement is likely due to mechani-
cal factors.

93
Chapter 4

the speed of the movement in the eye with limited Forced (passive ductions)
motility (the right eye). If this eye moves at a normal
saccadic speed (200 to 400 degrees/sec), as does the Forced or passive ductions should be carried out
normal eye which serves as a control, the apparently at some time on all patients undergoing strabismus
underacting muscle (the right lateral rectus) is proba- surgery. This test can be done at the time of surgery,
bly contracting in a normal or nearly normal way. after adequate anesthesia has been obtained. The test
The limited motility is probably caused by mechani- is performed in both eyes in all directions. In most
cal restriction associated with this muscle's antagonist cases, forced or passive ductions are performed in the
or other fascial structures around the globe. On the operating room just before the actual surgery is
other hand, if the eye moves to its final position in begun. The test is accurate immediately if a non-
attempted dextroversion with a slow, floating move- depolarizing muscle relaxant has been used during
ment ( 30 degrees/sec), which is slower than the fel- general anesthesia, but can only be performed after 15
low eye, this evidence suggests that the right lateral or 20 minutes if succinylcholine or equivalent has
rectus is paretic. In this case, little can be determined been used because of muscle contraction.
regarding the presence or absence of an associated In cooperative patients with restricted motility
mechanical restriction. A possible co-existing about whom the surgeon wants as much information
mechanical restriction is determined only after pas- as possible before going to the operating room, forced
sive duction testing has been performed. Saccadic ductions can be carried out in the office using topical
velocity analysis also can be performed with the aid anesthesia. Several drops of proparacaine hydrochlo-
of an electro-oculograph that provides a printed read- ride or tetracaine are sufficient to anesthetize the con-
out. The electro-oculograph measures peak velocity junctiva. In place of these anesthetic agents, 5% lido-
on one track. A second track measures the magnitude caine (Xylocaine) drops may be used. As another
of the ocular movement and displays a slope of the alternative for anesthesia, a cotton-tipped applicator
ocular movement speed (Figure 5). saturated with cocaine hydrochloride 4% is held
against the conjunctiva at the point where the forced

Figure 5
A Limited movement in the right eye with normal saccadic B A slow saccadic velocity indicates weakness of the right
velocity indicates that restriction is causing the strabis- lateral rectus muscle. No information is revealed about
mus. possible coexisting restrictions. This must be analyzed
with forced duction testing. Peak velocity is not shown.

94
Workup of the strabismus patient

duction forceps are to be applied. Fine-toothed for- Traction testing of the superior oblique tendon
ceps are used to grasp the conjunctiva and episclera, can reveal laxity of the tendon which is the hallmark
and the patient is asked to look toward the field of of anatomic congenital superior oblique palsy with
action of the restricted motility. The examiner then its frequent anatomic anomalies of the tendon. When
gently assists the eye into the full extent of the laxity of the superior oblique tendon is found, it is
attempted duction. much more likely that the superior oblique tendon
Three important techniques to practice when will be explored, found to be loose or abnormally
doing the forced ductions on a patient in the clinic are inserted, and subsequently tucked, resected, or redi-
1) gently lift the eye as it is rotated on the physiolog- rected. On the other hand, if the tendon is thought to
ic axis center of rotation while avoiding pushing the be normal based on the superior oblique traction test,
globe back in the orbit, 2) grasp the conjunctiva- epis- the diagnosis is more likely acquired superior oblique
clera with a secure bite with the forceps, and 3) palsy or neurogenic congenital superior oblique palsy
instruct/reassure the patient to continue looking in the and tuck or resection of the superior oblique is not
field to be tested and avoid a refixation that could performed, avoiding a postoperative Brown syn-
cause the cornea to be scraped by the forceps creating drome. This superior oblique traction test adds to the
a corneal abrasion or tearing the conjunctiva. accuracy of both diagnosis and treatment of superior
It has been suggested that passive ductions can oblique palsy. Saunders and later Plager described an
be tested using just a cotton-tipped applicator to move intraoperative traction test to determine the proper
the globe after instillation of a topical anesthetic. amount to tuck the superior oblique tendon. Guided
This technique avoids the complications just by this test the tuck can be loosened if the tendon is
described. found too tight or tightened if the tendon remains lax.
In the example shown, the patient has a right To perform the superior oblique traction test on
esotropia and limited abduction of the right eye the right eye, shown from above in Figure 7, the eye
(Figure 6). After anesthetizing the eyes the patient is is grasped at the limbus with fine-toothed forceps at
asked to look as far to the right as possible. The con- the 4 to 10 o'clock positions (shown) or the 2 and 8
junctiva and episclera of the right eye are grasped o'clock positions (not shown) on the left eye. The
with a fine-toothed forceps at the nasal limbus (3 view is from above the patient's head. The eye is
o'clock position). The examiner then attempts to pushed back in the orbit in full adduction. The eye is
abduct the right eye gently but forcibly while follow- then brought temporally while continuing to push it
ing the normal arc of rotation of the eye around its back in the orbit. A normal taut superior oblique will
physiologic vertical axis. If the eye cannot be abduct- cause the globe to pop up. This reaction can be felt
ed fully, a mechanical restriction is present and the and seen. As the globe slips over the superior oblique
limitation of abduction results from mechanical caus- tendon toward abduction, the eye recedes a bit further
es with or without associated paresis of the lateral in the orbit. If no tendon is felt or a very slack ten-
rectus (as inferred from saccadic velocity analysis). don is felt and the eye does not pop up but instead
If the eye can be abducted fully and the examiner slides back into the orbit when the globe is brought
feels no resistance to forced abduction, no mechani- temporally, the superior oblique is loose or absent.
cal restriction is present and a right lateral rectus pare- Regardless of the tightness or looseness of the tendon,
sis is indicated. This finding is always associated the eye is easily pushed back in the orbit in full
with a floating saccade. abduction. In the case of a very loose tendon the
cornea remains out of view during the temporal
Passive duction testing of the oblique excursion of the globe.
muscles Inferior oblique traction test
Superior oblique traction test The inferior oblique traction test is best used to
confirm that apparent inferior oblique overaction is
Guyton suggested an ingenious scheme for eval-
due to a taut inferior oblique muscle and is done when
uating the status of the superior oblique tendon. This
inferior oblique overaction persists after the muscle
test depends on feeling a knife edge response when
has ostensibly been weakened. When performing this
gently pushing the globe backward in the orbit while
test, it is not uncommon to find a tight inferior
rolling the globe back and forth over the stretched
oblique response in a muscle that has been previous-
superior oblique tendon. This test is interpreted
ly weakened. When this is encountered and the infe-
according to the surgeon's experience. It is performed
rior oblique is explored most likely either the cut ends
in the operating room with the patient under general
of the muscle have reunited or some other type of
anesthesia to achieve the proper level of muscle
fibrous attachment is effective in causing persistent
relaxation. Plager has expanded on the testing for
inferior oblique overaction. In either situation, sever-
tightness (or laxity) of the obliques, adding testing of
ing the attachments that cause inferior oblique func-
the inferior oblique muscle.
tion lateral to the inferior rectus is usually effective

95
Chapter 4

Figure 6
A Fixing with the sound left eye (primary deviation) D If the eye goes freely into abduction, passive or forced
B In dextroversion, limited abduction of the right eye is ductions are free confirming that no mechanical restric-
seen. tion is present.
C After topical anesthetic has been applied, the examiner
attempts to complete abduction of the right eye. If this
is not possible - the eye is stiff and immovable -
mechanical restriction is present.

96
Workup of the strabismus patient

Figure 7 The superior oblique traction test (viewed from above the patients head)
A The eye is grasped at the 2 oclock and 10 oclock posi- C With a lax or loose tendon the cornea disappears and
tion (right eye from above) in preparation for the superior remains hidden behind the upper lid as the eye is rotat-
oblique traction test. ed.
B The eye is pushed back into the orbit and is guided from D The relative path of the globe as it passes over a normal
nasal to temporal. As it goes over the normal superior tendon.
oblique tendon, the eye pops up. E A lax superior oblique tendon allows the globe to be
pushed backward into the orbit.

97
Chapter 4

provided that the case is not otherwise complicated. hydrochloride 4% held against the conjunctiva at the
In cases of inferior oblique adherence or inferior point where it is to be grasped with the forceps may
oblique inclusion, however, the outcome of reopera- also be used to anesthetize the conjunctiva. The anes-
tion is not so optimistic. Restrictions often persist in thetized conjunctiva and episclera of the right eye are
spite of careful attempts to free all adhesions in the grasped with fine-toothed forceps at the nasal limbus
infero-temporal quadrant. (3 o'clock) and the patient is asked to move the eye
To perform the inferior oblique traction test, the slowly far to the right while the examiner attempts to
eye is grasped at the limbus with fine-toothed forceps stabilize the right eye in extreme adduction. The
at the 2 and 8 o'clock positions on the right eye (or the amount of tug felt by the examiner through the for-
4 and 10 o'clock positions on the left eye), shown ceps indicates the contracting power of the right lat-
from above in Figure 8. The eye is pushed back in the eral rectus. If no appreciable tug is felt, little or no
orbit in full adduction. The eye is then brought tem- contraction of the right lateral rectus occurred. This
porally while continuing to push back in the orbit. A type of response is associated with a floating saccade.
normal or taut inferior oblique will cause the globe to If the examiner feels a brisk tug on the for-
pop up. This reaction can be felt and seen. If no ceps stabilizing the eye, the right lateral rectus has
taut muscle is felt and the eye does not pop up when significant contracting power. This type of response
the globe is brought temporally, the inferior oblique is is associated with a brisk, normal-velocity, saccadic
not tight and probably has been effectively weakened. movement. The limited movement in this case is
Regardless of the tightness or looseness of the mus- caused by a mechanical leash or tethering effect
cle, the eye is easily pushed back in the orbit in full usually caused by scar tissue, adhesions, or a spastic,
abduction. contracted antagonist. The surgeon can test generat-
ed force in the normal eye of a patient after topical
Generated muscle force anesthesia to establish a feel for this test.
Another step in analysis of strabismus with More accurate determination of active mus-
restricted motility is to perform the active muscle- cle force generation can be obtained by using a strain-
force generation test. This test determines, in the gauge. Black silk sutures (4-0 or 5-0) are affixed to
presence of restricted eye movements, the amount of the episclera and attached to the strain gauge deflec-
force generated by a given extraocular muscle within tor. A perilimbal suction cup or a special forceps can
the range of movement noted on testing of versions be used to connect the episclera to a suture or other
and ductions. Active muscle force generation is a tac- device that in turn attaches to the strain gauge.
tile test that complements saccadic velocity analysis Isometric contractions should be 60 to 90 gm or more
which is a visual test. in a normal muscle and are reduced to approximately
The information obtained from saccadic analy- 10 gm in complete paralysis. Even with paralysis
sis, forced ductions, the muscle-force generation and some force is measured because of passive tissue
in a few cases the differential intraocular pressure test forces. These more accurate tests are reserved for the
helps indicate if recession-resection and freeing of laboratory.
restrictions are indicated or if muscle transfer with or
without freeing of restrictions is required. When nor- Differential intraocular
mal contraction plus mechanical restriction is present, pressure test
the restriction first must be eliminated by freeing con- The differential intraocular pressure (IOP) test is
junctival-globe-muscle-fascial adhesions. Detaching a useful indirect technique for diagnosing generated
and recessing or otherwise weakening a muscle may muscle force and confirming mechanical restriction
be required. Either recession alone or a recession- in the face of a normal agonist. This test is based on
resection procedure should be performed as indicat- the fact that in the normal patient the eye rotates
ed. When reduced or weak muscle contraction is dis- around the center of the globe. During normal move-
covered, as evidenced by reduced generated muscle ment the antagonist relaxes as the agonist contracts
force, a muscle transfer is usually indicated. This is and no excessive pressure is placed externally on the
done with or without weakening the antagonist based globe, and IOP remains normal. At extremes of duc-
on the forced duction test, the age of the patient, and tions in the normal eye, an increase in IOP occurs
the number of undisturbed anterior ciliary arteries. because ocular movement is stopped by mechanical
To start the test, the patient is instructed to look checking by the antagonist rather than by lack of ago-
in the direction opposite from the field of action of the nist contraction power. In cases where ocular move-
muscle to be tested. In the example cited (a right ment is limited by mechanical restriction, the IOP
esotropia with limited abduction of the right eye), the increases when the eye attempts to move into the
patient is asked to look far to the left (Figure 9). restricted field. On the other hand, when a restricted
Several drops of proparacaine hydrochloride (0.5%) movement is not accompanied by an increase in IOP,
are sufficient to anesthetize the cornea and conjuncti- paresis may be indicated. The differential IOP test
va. A cotton-tipped applicator saturated with cocaine

98
Workup of the strabismus patient

A B

C1 C2

Figure 8 The inferior oblique traction test viewed from above the patients head
A The right eye is grasped at the limbus. C2 If no pop is felt, the inferior oblique has been effective-
B The eye is rotated nasally and pushed back in the orbit. ly weakened.
C1 The eye is brought temporally -- the surgeon feels for D The eye normally recedes in abduction regardless of
the pop up of the inferior oblique which is still present. the state of the inferior oblique.

99
Chapter 4

B1

a slight tug
signifies a weak
muscle

B2

brisk - firm tug


signifies a
normal muscle

Figure 9
A To test abducting force in the right lateral rectus, the B2 If the tug on the forceps is felt as strong then it confirms
right eye is treated with appropriate topical anesthesia that the muscle is innervated - not paralyzed.
and the patient is instructed to look in extreme left gaze. C If the examiner has difficulty fixing the eye nasally, the
B1 The right eye is grasped at the nasal limbus (or temporal restraining forceps can be placed temporally (9 oclock
limbus) with fine-toothed forceps. The patient is asked in the right eye). Great care must be exercised to avoid
to look slowly to far right gaze. If only a slight tug is felt losing the firm grasp of the eye that could result in
in the muscle tested (the right lateral rectus in this case), scratching the cornea. The tester should remain alert
a paresis or paralysis is confirmed. to pull the forceps away if the grip is loosening.

100
Workup of the strabismus patient

can be used in the evaluation of strabismus with ment is limited by a restriction, the point of restriction
restriction in a patient at any age, but it is especially becomes a fulcrum, the eye is pulled backward, and
useful in a patient who will not cooperate for other the IOP increases. A longstanding right esotropia
tests of generated muscle force. Patients with thyroid with normal lateral rectus function in the right eye but
ophthalmopathy especially involving the inferior rec- restriction of the antagonist right medial rectus is
tus often have chronic elevation of intraocular pres- shown. This condition can occur after recovery from
sure by this mechanism. Optic nerve changes and a sixth nerve palsy or with co-contraction in Duane.
visual field defects characteristic of glaucoma have Orbital resistance meeting the backward-pulled eye
been seen in such cases. produces an elevation in IOP, as recorded earlier in
The eye normally rotates around its center, the the primary position. If in the cases cited previously
antagonist relaxing and the agonist contracting with the limited abduction of the right eye were due to
no increase in IOP until the extremes of duction have paresis of the right lateral rectus, no rise in IOP would
been encountered (Figure 10). When an ocular move- occur on attempted abduction.

A
15 mmHg 15 mmHg

Figure 10
A As the eye rotates in adduction around the Z axis no B As the eye attempts to rotate in adduction a stiff, non-
additional pressure is exerted and intraocular pressure is relaxing lateral rectus becomes a fulcrum, the globe
unchanged. retracts and pressure rises.
continued.

101
Chapter 4

C1

C2

Figure 10, contd


C1 Intraocular pressure increases during attempted abduc- D In the absence of force toward abduction with or without
tion in the right eye that has normal or near normal associated restriction, no increase in pressure occurs.
force generated in the lateral rectus in the presence of
restriction to abduction.
C2 Force on the globe as it is pulled back against the
orbital contents.

102
Workup of the strabismus patient

muscle insertions on the globe, the muscle's effect on


A case posted on the pediatric ophthalmology movement, and considering potential passive
list serve described a 49-year-old man who had mechanical restrictions to movement.
a large left exotropia and limited adduction. On The globe moves around the x-, y-, and z-axes as
attempted adduction of the left eye intraocular shown in Figure 11. The eye abducts and adducts
pressure increased from 15 to 55 mmHg. The around the z-axis, elevates and depresses around the
optic nerve demonstrated glaucomatous cupping x-axis, and carries out intorsion and extorsion around
and a typical glaucoma visual field defect was the y-axis. The eye moves according to a combina-
found. In this case, release of the restriction tion of these factors in Listings plane but for practi-
would relieve the increased pressure and slow cal purposes, it is useful to think of these movements
or eliminate the glaucomatous process. in isolation. The globes are shown in Figure 12 as
viewed from above. They are divided into quadrants.
The superior rectus (SR) elevates, adducts, and intorts
the globe. The superior oblique (SO) depresses,
Motor physiology - The muscles
actions
After the patient's ocular motility evaluation has
been completed a decision is made which may
include planning for extraocular muscle surgery. This
plan for surgical treatment should be written on the
patient's record when the patient is scheduled for sur-
gery. In most instances, the specific muscles to be
operated and the direction and amount they are to be
weakened, strengthened, or shifted will have been
decided. This plan is not usually influenced by the
alignment of the eyes in the operating room after
anesthesia has been obtained. However, in those
patients with restricted motility, particularly those
who have been operated on previously, the type and
amount of surgery can be determined only after per-
forming forced ductions while the patient is asleep
and in some cases only after assessing the state of the
muscles and associated restrictive fascia seen during
dissection at the time of surgery.
When recording a plan for surgery, it is helpful to
think in terms of the mechanical components. This
may be accomplished by picturing the location of the
Figure 11 Ficks Axes

Figure 12
The superior oblique depresses, intorts and abducts. The superior rectus elevates,
adducts, and intorts.

103
Chapter 4

abducts, and intorts the globe. In addition, the anteri- When a horizontal rectus muscle is shifted verti-
or fibers of the superior oblique tendon are primarily cally, this muscle has less effect on the globe align-
responsible for intorsion and the posterior fibers for ment in the same direction as the muscle is shifted.
depression. If the superior oblique tendon is moved For example, when the medial rectus is shifted up,
forward, it will increase intorsion. If the superior rec- this muscle has less adducting effect in upgaze.
tus is moved temporally, it will increase intorsion, When the medial rectus is shifted downward, adduct-
and if it is moved nasally, it will decrease intorsion. ing effect is less in down gaze. The same applies for
Both of these superior muscles move the globe in the the lateral rectus. This response is the basis of verti-
x-, y-, and z-axes. cal shift of the horizontal recti to treat A or V pat-
The globes are shown as viewed from below in tern.
Figure 13. The inferior rectus (IR) depresses, As a rule, all previously operated muscles that
adducts, and extorts the globe. Recessing the inferior are being considered for surgery should be inspected
rectus as done in thyroid restrictive disease can result under direct visualization before any strengthening,
in decreased adduction and an A pattern. This can be weakening, or transfer procedures are performed. In
avoided by a nasal shift. The inferior oblique (IO) ele- case of reoperation, findings at the time of surgery
vates, abducts, and extorts the globe. Both of these could lead to change in the surgical plan. For exam-
inferior muscles move the globe in the x-, y-, and z- ple, in a patient with secondary exotropia occurring
axes. after recession of the medial rectus and resection of
In the primary position, the medial rectus (MR) the lateral rectus for esotropia, depending on the
and lateral rectus (LR) muscles adduct and abduct the angles, the lateral rectus would require weakening
globe, respectively (Figure 14). The eye is viewed and the medial rectus strengthening. If treated like a
from the lateral aspect. These muscles move the new case, the lateral rectus would be weakened first
globe around the z-axis. With the eye elevated, both and then the medial rectus strengthened. This
horizontal rectus muscles elevate the globe (Figure approach follows the rule of doing the recession first
15). This adds movement of the globe around the x- in a recession-resection procedure. However, in sec-
axis. With the eye depressed, both horizontal rectus ondary cases where two muscles will be operated and
muscles depress the globe. Both horizontal rectus forced ductions are unrestricted and ductions limited,
muscles can be shifted upward to improve elevation the muscle to be strengthened is isolated first and
or downward to improve depression (Figure 16). tagged with a 4-0 silk suture. This is a good practice
When the medial or lateral rectus insertion is shifted to follow because it allows the surgeon to determine
upward, the muscle becomes an elevator in part. if a previously operated muscle is indeed present or,
When the medial or lateral rectus insertion is shifted if present, has slipped from the intended point of
downward, the muscle becomes a depressor in part. insertion. The condition of the muscle to be strength-
Mechanical restrictions hampering ocular move- ened by advancement and resection may influence the
ments can be associated with the following: (1) con- amount of weakening that should be done on the
junctiva, (2) anterior Tenon's capsule, (3) the muscle antagonist, if any at all, or may indicate that a muscle
itself, (4) intermuscular membrane, and (5) orbital fat transfer should be performed. For example, esotropia
(Figure 17). All of these factors must be considered occurring after recession of the lateral rectus and
during surgery. resection of the medial rectus for exotropia and with

Figure 13
The inferior oblique elevates, extorts, and abducts. The inferior rectus depresses,
adducts, and extorts.

104
Workup of the strabismus patient

limited forced (and voluntary) abduction may be


treated with recession of a tight medial rectus alone
(provided muscle-force generation of the lateral rec-
tus is adequate). In reoperations it is essential to
assess the relative roles played by weak or slipped
muscles, tight muscles or adhesions, or both. In
these cases surgery on a single muscle may be suffi-
cient if significant muscle slippage or restriction is
discovered.
Figure 14 Herings law - yoke muscles
The lateral rectus abducts and the medial
rectus adducts. The extraocular muscles are yoked in their action
according to Hering's law of distributed innervation.
By this law, muscles in each eye are associated as by
a yoke (as oxen in days of old and in some parts of the
world now) and act together to move the eyes toward
the object of regard while receiving equal innerva-
tion. Movements of the eyes together by the action of
yoked muscles are called versions. This is in contrast
to ductions or movements of just one eye. Surgical
alteration of one extraocular muscle in the fixing eye
Figure 15 always affects its yoked muscle. This must be con-
In upgaze the horizontal recti may con-
tribute to elevation. sidered in every patient undergoing extraocular mus-
cle surgery. For example, deficient elevation of the
right eye in adduction can be caused by underaction
of the right inferior oblique muscle. If the surgeon
wishes to enhance elevation of the right eye in adduc-
tion but does not wish to carry out a strengthening
procedure on the right inferior oblique (not an easy or
straightforward procedure), the yoke muscle of the
right inferior oblique - the left superior rectus muscle
- may be weakened. In this case the left eye will
Figure 16 require more innervation to elevate in abduction. By
In downgaze the horizontal recti may con-
tribute to depression.

Figure 17
Mechanical restriction limiting eye movement can be association with:
1 Conjunctiva 4 Intermuscular membrane (posterior Tenons)
2 Anterior Tenons 5 Orbital fat
3 The muscle

105
Chapter 4

A B

C D

E F

G H

Figure 18 The Yoked Extraocular Muscles


A The right lateral rectus and left medial rectus muscles F The right superior rectus and the left inferior oblique
are yoked to produce dextroversion. muscles are yoked to produce elevation in up right gaze.
B The left lateral rectus and right medial rectus muscles G With the head tilted 45 right, the right superior oblique
are yoked to produce levoversion. and right superior rectus rotate the right eye clockwise.
C The right inferior oblique and the left superior rectus In the left eye, the left inferior oblique and left inferior
muscles are yoked to produce elevation in up left gaze. rectus are clockwise rotators.
D The right superior oblique and left inferior rectus mus- H With the head tilted 45 left the left superior oblique and
cles are yoked to produce depression in down left gaze. left superior rectus rotate the left eye counter clockwise
E The right inferior rectus and left superior oblique mus- and the right inferior oblique and right inferior rectus are
cles are yoked to produce depression in down right counter clockwise rotators.
gaze.

106
Workup of the strabismus patient

Hering's law, the yoke of the muscle responsible for test that sorts out paretic obliques and vertical recti
elevation in abduction (left superior rectus) which is (Figure 19).
the right inferior oblique will receive more innerva- During upgaze the inferior oblique muscles can
tion. By this technique the right inferior oblique can overact causing a V pattern. The abducting force of
be strengthened. If in this example the eye with the the inferior obliques producing a greater exodeviation
paretic muscle is used for fixation, weakening the in upgaze results from weakness of the superior
yoke will simply move the fellow eye down to align obliques, upward displacement of the medial pulleys
with the eye with the underacting muscle. This prin- or weakness of adduction after recession of the medi-
ciple is applied with the posterior fixation suture to al recti. Antimongoloid fissures are common in V
produce a laudable secondary deviation. esotropia (Figure 20).
In addition to the six positions of gaze testing In downgaze, overaction of the superior obliques
the action of these yoked muscles, the alignment of results in an A pattern that is seen in both exo and
the eyes is observed in primary position with the head esodeviations. Overaction of the superior obliques is
tilted 45 to the right and the left, and looking 30 up seen with mongoloid fissures and with downward dis-
and 30 down. placement of the medial pulleys.
The fact that the oblique and rectus muscles are As mentioned previously, non-surgical treatment
torsional synergists but vertical antagonists is the will not be discussed in detail in this text. All appro-
basis for the Bielschowsky head tilt test which priate nonsurgical treatment including glasses,
demonstrates greater vertical deviation when the head prisms, anticholinesterase, occlusion, orthoptics, and
is tilted toward the side of the paretic superior the like should be carried out in appropriate cases
oblique. This is also the key step in the Parks 3-step before embarking on surgery.

A Parks 1

B Parks 2

Helveston Step I In a case with a hyperdeviation, the adducted eye in the lateroversion of greater vertical deviation
points to the ipsilateral oblique and contralateral rectus as the possibly paretic muscle.

C Parks 3

Helveston Step II If the vertical deviation is greater with head tilt toward the higher eye, the oblique from Step I is paret-
ic. If the opposite is true; that is, the vertical deviation is greater with the head tilted toward the lower eye, the vertical rec-
tus from Step I is paretic.

Figure 19 Positive Bielschowksy test in a patient with right superior oblique palsy
A Right hypertropia shown here latent because of vertical right superior rectus as it responds to the need to intort the
fusional amplitudes right eye. This is shown here as the Parks Three Step
B Overaction of the right inferior oblique in levoversion Test.
C With head tilt to the right the hypertropia increases
because of the weakly opposed elevating action of the

107
Chapter 4

B C

D E

Figure 20
A Antimongoloid fissures are associated with V pattern C Looking down the abducting action of the superior
esotropia. Mongoloid fissures are associated with A oblique can produce an A.
esotropia. Esotropia in this patient is in upgaze only. D Lax superior oblique tendons, upward displacement of
This rule does not necessarily hold for exodeviations. pulleys and recessed medial rectus contribute to a V.
B Looking up the abducting action of the inferior obliques E Downward displacement of the pulleys contribute to an
can produce a V. A.

Step 2: Results to be expected Horizontal rectus surgery for


from surgery esotropia
Each surgical procedure done on the extraocular
muscles is unique. This surgery is influenced by fac-
tors related both to the patient and to the specific sur-
gical procedure performed. These issues notwith- Single muscle procedure for esotropia:
standing, it is possible to establish guidelines that can Surgery on a single horizontal rectus muscle as
be useful in initial planning and proper execution of primary treatment of esotropia should be employed
successful strabismus surgery. The guidelines pre- sparingly and then only for special reasons. A special
sented here are only a launching pad for the surgeon reasons might be a patient with small-angle esotropia
who will gain experience in techniques of strabismus and fusion potential where a single medial rectus
surgery. Once a surgical career has taken flight, each recession could promote fusion. A single medial rec-
strabismus surgeon must apply his or her own valid tus recession is usually performed because the patient
personal modifications to the following. has diplopia and/or asthenopia and has potential for
bifoveal fusion.

108
Workup of the strabismus patient

Individuals with esotropia, who have an angle Upward displacement of the medial pulleys or
sufficiently small to be corrected by a single medial downward displacement of the lateral pulleys or con-
rectus recession, tend to have peripheral fusion and genital laxity of the superior oblique tendons are also
harmonious anomalous retinal correspondence and a likely cause of a V pattern. In either case, the infe-
are included in the monofixation syndrome. Such rior oblique may apparently overact but the real
patients are usually cosmetically acceptable and are cause for hyperdeviation in adduction and for abduc-
often better without surgery. A single medial rectus tion in elevation (producing the V) is deficient
recession performed for fear of producing an over- checking with a lax tendon or shifted vectors with
correction is usually a manifestation of trepidation on pulley displacement.
the part of an overcautious surgeon. Single medial The desire for symmetry is not necessarily an
rectus recession for patients with limitation of motili- indication for performing a bimedial rectus recession
ty such as Duane syndrome is worthwhile and is dis- in every case of esotropia. A recession of the medial
cussed elsewhere (see chapter 5). rectus and resection of the lateral rectus is indicated
Resection of a single lateral rectus muscle for in cases with poor vision in one eye or with other rea-
esotropia is less effective than recession of a single sons to limit surgery to one eye. In addition, some
medial rectus muscle and is less likely to be indicated surgeons simply prefer to perform recession-resection
except in specific cases such as a lateral rectus mus- and this is perfectly acceptable.
cle that has slipped after prior surgery. Advancement Bimedial rectus recession measured from the
of a previously recessed lateral rectus can be effective limbus. Since 1975 I have measured all bimedial rec-
in cases of small angle incomitant esotropia with lim- tus recessions using the limbus as the reference point.
ited abduction after lateral rectus recession. This is done for two reasons. First, the medial rectus
insertion site was found to be extremely variable,
ranging from 3.0 to 6.0 mm (average 4.4 mm) in a
series of esotropic patients, with the insertion site
having no relationship to the angle of deviation.
Two muscle surgery for esotropia: Second, up to 50% of patients undergoing bimedial
rectus recession for congenital esotropia before this
Bimedial rectus recession measured from the
time (when maximum medial rectus recession was
insertion. A minimal bimedial rectus recession of 2.5
5.0 to 5.5 mm) required additional surgery because of
mm reduces an esodeviation approximately 15 to
undercorrection of the esodeviation. The unaccept-
20. A maximum bimedial rectus recession of 7
able number of undercorrections suggested that a
mm* results in as much as 40 or more reduction in
larger bimedial rectus recession should be performed
the esodeviation. Slightly more effect may be
provided it could be accomplished safely.
obtained in infants, but definitely less effect is pro-
Rationale for measuring from the limbus.
duced in adults. Indications for bimedial rectus reces-
Assuming that the medial rectus muscles could be
sion include:
recessed to the equator without crippling the action of
1. Congenital esotropia up to 50
the muscle, we decided to move the medial rectus
2. Esotropia in an adult up to 40
muscle to the equator and consider this a maximum
3. Equal vision
recession for large-angle congenital esotropia. The
4. Esotropia greater at near (high AC/A)
medial recti could then be recessed a lesser amount
5. Excess adduction
for smaller angles while still measuring from the lim-
A bimedial rectus recession with vertical shift
bus. The easiest way to locate the equator is to use
may also be performed in patients with an A or V pat-
the limbus as a reference. Since the corneal diameter
tern, especially in patients without oblique dysfunc-
defined by the limbus as well as the axial length are
tion. Lack of oblique muscle overaction is determined
fairly consistent according to patient age, it is rela-
by noting absence of hyper- or hypo-deviation in the
tively easy to calculate the distance of the equator
adducting eye in latero-version. In this case, the
from the limbus. In the infant between six months
medial rectus muscles are shifted vertically toward
and one year, this value is approximately 10.5 mm.
the closed end of the pattern. This means that the
Between four and six months the maximum is 10.0
recessed medial rectus muscles are shifted upward for
mm. In the child over one year, the equator is approx-
an A pattern and downward for a V pattern.
imately 11.5 mm from the limbus. Therefore, these
In patients with a V pattern with inferior
dimensions were utilized as guides for performing
oblique overaction, a bimedial rectus recession and
bimedial rectus recessions. These maximum reces-
bilateral inferior oblique weakening is usually the
sions have been used for any large angle of congeni-
preferred technique.
tal esotropia, even those in excess of 50. On the

* This might be excessive in a normal or smaller eye with a medial rectus insertion 5.5 mm from the limbus.

109
Chapter 4

Less than 1 year More than 1 year

Medial rectus recession Medial rectus recession


ET ET
measured from limbus measured from limbus

25 8.5 mm 25 8.5 mm

35 9.5 mm 35 9.5 mm

45+ 10.5 mm 45 10.5 mm

< 6 mo 10.0 mm 55+ 11.5 mm

Table 1

other hand, smaller deviations receive smaller reces- cases without tight conjunctiva, and this includes
sions with a minimum bimedial rectus recession for most cases with congenital esotropia, I use a cul-de-
congenital esotropia being 8.5 mm from the limbus. sac incision.
Intermediate deviations are titrated between these
numbers and are described above (Table 1). It should
be emphasized that these numbers are merely guide-
lines. The surgeon should individualize his/her surgi-
cal numbers according to experience.
Measuring from the limbus has proved to be an Bilateral lateral rectus resection. I seldom per-
effective way of performing a larger bimedial rectus form bilateral lateral rectus resection. It is used most-
recession without crippling the effect of the medial ly as a second procedure by surgeons who routinely
rectus muscle postoperatively. This technique has do a smaller bimedial rectus recession as an initial
resulted in 80% to 85% of patients being aligned sat- procedure for large-angle congenital esotropia and
isfactorily after just one procedure. Other surgeons who decline re-recession of the medial rectus mus-
have equally good results performing bimedial rectus cles. As a rule, a strengthening procedure of a rectus
recessions of up to 7 mm (rarely more) measuring muscle without recession of its antagonist at the same
from the original insertion. However, I continue to procedure is less effective at reducing the angle of
use measurement from the limbus because of the strabismus than a weakening procedure without
wide variations in the medial rectus insertion site and strengthening of the antagonist. Resection is consid-
because of the excellent results obtained with limbal erably less effective when done alone than the same
measurements. resection combined with a recession of the antagonist
When bimedial rectus recession measured from performed at the same procedure. Two situations that
the limbus was introduced, it included routine con- call for bilateral lateral rectus resection are diver-
junctival recession and was called the en bloc or aug- gence insufficiency (paralysis) and residual esotropia
mented recession. Analysis of results indicated that it in a patient who has undergone a maximum bimedial
was unnecessary to routinely recess the conjunctiva. rectus recession. Approximately 20 of esodeviation
We now perform conjunctival recession to the origi- is corrected with a minimal 5 mm bilateral lateral rec-
nal medial rectus insertion site when performing a tus resection and although good data are lacking, I
bimedial rectus recession only in cases where passive estimate that up to 35 to 40 of esotropia could be
abduction is limited preoperatively by a tight con- corrected with a maximum 9 to 10+ mm bilateral lat-
junctiva that occurs in approximately 5% of cases. In eral rectus resection.

110
Workup of the strabismus patient

that might remain. However, residual esotropia in


this case could be treated with a marginal myotomy of
a previously maximally recessed medial rectus mus-
cle combined with a resection of the unoperated lat-
eral rectus muscle. This technique can, in my experi-
Recession of the medial rectus - resection of the
ence, be effective treatment for residual esotropia
lateral rectus. A minimum recession-resection pro-
after previous three muscle surgery. However, the
cedure for esotropia is 2.5 mm medial rectus reces-
surgeon must remain alert for specific reasons why a
sion and a 5 mm lateral rectus resection that could be
patient does not respond in the way expected. Is
expected to correct 20 to 25 of esotropia. But, if a
unrecognized restriction, paralysis, or misdirection
smaller angle is treated, a single medial rectus reces-
the cause? Is further evaluation required?
sion could be considered. A maximum recession-
resection procedure of 5 mm(10.5 mm from the lim-
bus) medial rectus recession and 9 mm lateral rectus
resection in a child less than 1 year of age and of 7
mm (11.5 mm from the limbus) medial rectus reces-
sion and 10+ mm lateral rectus resection in a child
over 3 years corrects up to 50+ of esotropia. Four muscle surgery for esotropia:
Increasing the minimum numbers or decreasing the Bimedial rectus recession - bilateral lateral rec-
maximum numbers can be done for deviations tus resection. Esotropia greater than 75 may be
between 20 and 50 of esotropia and for children treated surgically with a bilateral recession-resection
between the ages of 1 and 3 years. procedure. This four muscle procedure should be
The amount of surgery may be divided between used with discretion and is not often indicated. Four
the medial and lateral rectus muscles according to muscle surgery should not be performed, except in
findings on ductions and versions as well as on dif- special circumstances, in infants. However, certain
ferences between distance and near measurements. If adults and older children with esodeviations of
excess adduction and/or greater deviation at near is between 75 and 100, particularly with limited
found, more emphasis is placed on the medial rectus forced abduction usually from longstanding strabis-
recession. If deficient abduction and/or greater devi- mus can benefit from a bimedial 7 mm rectus reces-
ation in the distance is present, more emphasis is sion (11.5 mm from the limbus) and a 10 mm bilater-
placed on the lateral rectus resection. This represents al lateral rectus resection. Four muscle surgery
the symmetrizing effect of the recession-resection should ordinarily be limited to these maximum
procedure. numbers.

Horizontal rectus surgery for


exotropia

Three muscle surgery for esotropia:


Bimedial rectus recession and lateral rectus Single muscle surgery for exotropia:
resection. When more than 50 of esotropia must be Single lateral rectus recession or single medial
corrected, some surgeons believe strongly that sur- rectus resection. A single lateral rectus recession or
gery should be performed on three horizontal rectus a single medial rectus resection for treatment of
muscles. This approach with maximum numbers exotropia is rarely indicated. However, certain
could correct up to 75 of esotropia, especially in a patients who have a very small incomitant exotropia
younger patient. This maximum procedure consists and who have fusion potential, often with head turn,
of a 5.0 mm bimedial rectus recession and 9 mm can be helped by this procedure. These patients are
resection of one lateral rectus in patients less than 1 rare. In my experience, this condition is usually
year of age and a bimedial 7 mm (11.5 mm from the caused by insufficient initial surgery in which only
limbus) rectus recession and 10+ mm resection of one one lateral rectus muscle was recessed. In such a
lateral rectus in patients over 3 years of age. case, either the medial rectus should be resected or
Deviations between 50 and 75 are corrected by the lateral rectus recessed to produce the most nearly
reducing maximum three muscle surgery by 0.5 to 1 comitant results. The findings on versions and duc-
mm per muscle. Three muscle surgery for esotropia tions, as well as prism and cover measurements in the
had been criticized on the grounds that after such sur- lateral versions, indicate what need be done to
gery only one unoperated horizontal rectus muscle achieve comitance. For example, a tight lateral rectus
remains. Under these conditions, it has been said that after a recession and resection for esotropia or a case
it is more difficult to correct any residual esodeviation

111
Chapter 4

of exotropic Duane syndrome might be treated with a approximately 20 to 25 of exotropia and produce
single lateral rectus recession to relieve a small angle about the same reduction in the exotropia in the dis-
of exotropia. Usually no more than 15 of deviation tance and at near. A maximum recession-resection
can be corrected with single muscle surgery for procedure for exotropia is 8 to 10 mm lateral rectus
exotropia. An exception would be a larger correction recession and 10+ mm medial rectus resection. This
obtained in case of advancement of a slipped muscle. procedure would be expected to correct up to 50 of
exotropia.
The majority of exotropic patients, in my experi-
ence, have basic exotropia (same exotropia distance
and near) or simulated divergence excess exotropia
Two-muscle surgery for exotropia: (near exotropia equal to or nearly equal to distance
exotropia after several hours occlusion of one eye).
Bilateral lateral rectus recession. A minimum
For this reason, a recession-resection procedure is
bilateral lateral rectus recession of 5 mm will correct
arguably the most logical procedure. However, to
approximately 20 to 25 of exotropia. A maximum
avoid the necessity of resecting the medial rectus
bilateral lateral rectus recession of 8+ mm can correct
muscle, a procedure which may produce a reddened
up to 50 of exotropia. The same angle would be cor-
conjunctiva and sometimes limited abduction, it is
rected if the deviation were either manifest or latent.
common for the surgeon to choose a bilateral lateral
Bilateral lateral rectus recession is a common strabis-
rectus recession in most cases of intermittent
mus surgical procedure.
exotropia.
A recession-resection procedure is an effective
and predictable way to alter the alignment of the eyes
to produce cosmetic as well as functional improve-
ment. Surgically induced incomitance is infrequent
and is not usually significant when it does occur. This
Bimedial rectus resection. Strengthening and
incomitance can be of benefit for patients treated for
weakening procedures of the extraocular muscles
convergence insufficiency because it allows them to
mainly improve ocular alignment while maintaining
find an area of fusion at near and at distance, even
or creating comitance. These procedures do not ordi-
when a small overcorrection occurs, by assuming a
narily influence vergences. Nevertheless, certain
face-turn.
patients demonstrating intractable convergence
insufficiency not helped by near point exercises or
other orthoptic treatment may be helped some with a
bimedial rectus resection. A 5 to 7 mm bimedial rec-
tus resection can be considered reasonable treatment
for a convergence insufficiency measuring between Three-muscle surgery for exotropia:
12 and 25 of exotropia at near and with less or no A maximum bilateral lateral rectus recession of 8
exotropia in the distance and with a remote near point to 10 mm combined with a maximum medial rectus
of convergence. Bimedial resection for convergence resection of 10+ mm in one eye will correct up to 75
insufficiency can result in esotropia in the distance as of exotropia. To correct deviations between 50 and
the price for relieving near symptoms. 75 maximum, three muscle surgery is reduced by
Intermittent exotropia persisting after bilateral 0.5 to 1 mm per muscle.
lateral rectus recession with a significant exodevia-
tion at distance and at near can also be treated with a
bimedial rectus resection. In such a case, a minimum
5 mm bimedial rectus resection can correct approxi-
mately 20 of exotropia. A maximum 10+ mm bilat-
eral medial rectus resection can correct up to 40 of Four-muscle surgery for exotropia:
exotropia.
A maximum 8 to 10 mm bilateral lateral rectus
recession combined with a maximum 10+ mm bime-
dial rectus recession will correct 90 to 100 of
exotropia. If four muscle surgery is indicated for
exotropia, a maximum approach is usually done.
Lateral rectus recession with medial rectus resec-
tion. A minimum recession-resection procedure for
exotropia is 5 mm lateral rectus recession and 5 mm
medial rectus resection. This approach will correct

112
Workup of the strabismus patient

Vertical rectus surgery


vious surgery. In selected cases, the inferior rectus
can be resected up to 10 mm and recessed up to 10
mm or even more. With recession of the inferior rec-
tus, the intermuscular membrane should be dissected
to at least 10 mm from the insertion and Lockwood's
ligament can also be brought forward to avoid lower
lid ptosis.
I have performed a free tenotomy of the inferi-
or rectus with minimal dissection of the intermuscu-
lar membrane in patients with severe thyroid myopa-
thy affecting the inferior rectus. This has produced
marked improvement in motility. A drawback is the
ptosis of the lower lid resulting in 2 to 3 mm widen-
ing of the palpebral fissure. This lower lid ptosis is
less objectionable if the procedure is done bilaterally.
These large recessions may be carried out more pre-
Single muscle surgery on the vertical rectus mus- dictably using an adjustable suture. However, the
cles can be effective and predictable in most cases. inferior rectus is susceptible to early or late slippage
This is in contrast to single muscle surgery on the hor- resulting in overcorrection because of the destabiliz-
izontal rectus muscles which is less effective and less ing effect of the inferior oblique, Lockwoods, and
predictable. A minimum 2.5 mm recession or resec- the lower lid retractors.
tion of either the superior or inferior rectus will pro- I have performed 9 mm resections of the inferior
duce approximately 8 of deviation reduction in the rectus without causing significant narrowing of the
primary position. This results in slightly more cor- palpebral fissure. It should be emphasized, however,
rection in the field of action of the muscle after reces- that patients should be selected very carefully before
sion and in the field of action of the antagonist after exceeding the usual limits of recession and resection
resection. A 5 mm recession or resection of a vertical on the inferior rectus. The superior rectus should not
rectus muscle will produce up to 15 of reduction in ordinarily be resected more than 5 mm. Ptosis
the primary position deviation and slightly more in involving the upper lid is easier to produce, harder to
the field of action of the muscle or of the antagonist. avoid, and cosmetically more objectionable than sim-
ilar displacement of the lower lid.
If the surgeon takes care when dissecting the
intermuscular membrane and does not cut the superi-
or oblique tendon, the superior rectus may be
recessed 10 mm or more without causing bothersome
retraction of the upper lid. When this very large supe-
rior rectus recession is carried out, it is usually for
treatment of dissociated vertical deviation. If the
superior rectus muscle is recessed more than 6 to 8
mm, theoretically it must be placed behind the supe-
rior oblique tendon as it passes beneath the superior
rectus muscle between the insertion of the superior
oblique and the trochlea. Most surgeons who claim to
perform large superior rectus recessions do so with
the hang-loose procedure. However, it cannot be
guaranteed that the muscle will adhere to a given spot
on the globe when a hang-loose suture is used.
However, those surgeons who prefer this technique
A combined recession-resection of the vertical recti claim to have confirmed satisfactory position of the
corrects with minimum numbers 15 and with maxi- recessed superior rectus at reoperation. When recess-
mum numbers 25 to 30 of deviation in the primary ing a rectus muscle, I prefer to suture the muscle to
position. The normal maximums for recession and the globe at the determined spot to assure accurate
resection of the inferior rectus can be exceeded in postoperative muscle placement. However, Repka,
patients who demonstrate restriction on testing with et. al. have shown reliable muscle placement after
forced ductions from such causes as thyroid myopa- hang-loose recession of the horizontal recti.
thy, old blowout fractures, fibrosis syndrome, or pre-

113
Chapter 4

Surgery of the oblique muscles superior oblique that should be tucked depends on
how lax or redundant the tendon is at the time of sur-
gery. This is determined by the superior oblique trac-
tion test (see page 95). This test should be done in
both eyes during every procedure for superior oblique
over or under action. It is done even if other muscles
are treated. During surgery the tendon is brought up
into the tucker or it is tucked free hand until the ten-
don feels taut; at this point the tuck is secured but
with a loop that allows for adjustment. The superior
oblique traction test is then repeated. The tuck is then
Superior oblique weakening: adjusted, if needed, and secured when the traction test
Tenotomy of the superior oblique muscle pro- is equal to or slightly tighter in the treated compared
duces approximately 5 to 15 reduction in the to the normal fellow eye. The size of the tuck may
hypodeviation in the primary position and slightly vary from 6 to 20 mm or more, but it is always dic-
greater reduction in the field of action of the muscle. tated by the preoperative tendon laxity and the
This procedure may be graded somewhat by shifting desired result is an equal or nearly equal superior
the site of the tenotomy closer to the insertion for less oblique traction test. Tuck of a superior oblique ten-
effect or closer to the trochlea for more effect. It may don done in the presence of a normal or tight traction
also be graded with a guard suture which in effect test preoperatively or performing too large a tuck of a
produces a hang-loose tendon lengthening. This was lax tendon will result in an unwanted iatrogenic
called a chicken suture by Phil Knapp. A hang Brown.
loose superior oblique tendon weakening can also be Resection of the superior oblique tendon may be
done from the insertion. If a tenectomy is done, the accomplished at the insertion in a similar amount to
degree of weakening of the superior oblique is prob- the tuck. In our experience, an effective superior
ably not affected by the amount of tendon removed oblique tuck or resection produces some Brown syn-
but instead by the proximity of the nasal margin of the drome or mechanical restriction to elevation in
tenectomy to the trochlea. The fascia in the vicinity adduction, at least in the early postoperative period.
of the superior oblique tendon should be left as undis- Strengthening of the superior oblique is usually per-
turbed as possible when doing a superior oblique formed in superior oblique palsy of the type which
tenotomy in order to achieve more predictable results. has recently been re-classified as congenital versus
In practice, unilateral superior oblique weakening is acquired. Of these two, the congenital palsies have
seldom performed except in the case of Brown syn- been shown to have a high rate of abnormalities of the
drome or superior oblique myokymia. Wright tendon. In most cases these are seen as a redundant
devised a system for weakening the superior oblique tendon. With a loose tendon, a tuck or any other
employing a silicone spacer. Bilateral superior strengthening procedure is safe and effective (see
oblique weakening is more common and is discussed chapter 16).
in the section on surgery for A and V patterns (see Anterior shift of the superior oblique:
chapter 16). In practice, bilateral weakening of the The intorting power of the superior oblique may
superior oblique tends to have greater effect in larger be enhanced by moving all or part of the effective
deviations and less effect in smaller deviations. insertion anteriorly and temporally. This is called the
Harada-Ito procedure. In my experience, superior
oblique tuck or resection improves both the torsional
and vertical deviation. However, when a large tor-
sional deviation of 10 to 15 degrees is present in a
patient who has a small vertical deviation, anterior
shift is a useful surgical tool. Anterior shift has been
done with an adjustable suture technique but this has
not become a popular procedure. Selective disinser-
tion of posterior tendon fibers weakens the depressor
Superior oblique strengthening: effect of the superior oblique, while selective disin-
Strengthening of the superior oblique by a tuck at sertion of the anterior fibers weakens intorsion.
its insertion produces up to 15 reduction in the
hyperdeviation in the primary position and a similar
reduction in the hyperdeviation in the field of action
of this muscle if the tuck is done properly producing
normal symmetric tendon tension. The amount of the

114
Workup of the strabismus patient

Surgery for vertically incomitant


horizontal strabismus (A and V
patterns)
A more or less fixed amount of surgery is per-
formed to treat vertical incomitance. This is accom-
Inferior oblique weakening: plished by shifting the horizontal rectus insertions
Weakening of the inferior oblique muscle results appropriately upward or downward or by weakening
in 5 to 10 reduction in hyperdeviation in the pri- overacting oblique muscles.
mary position and up to 20 less hyperdeviation in
the field of action of this muscle. Inferior oblique
weakening procedures may be graded, but the value
of this is not firmly established. When a recession is
performed it is usually + 8 mm. Disinsertion merely
frees the muscle from its insertion. With a myectomy,
a 5 to 8 mm segment of muscle is excised a few mil-
limeters below the inferior border of the lateral rectus Bilateral inferior oblique myectomy for treating
in the inferior temporal quadrant. Marginal or incom- a V pattern produces on the average 20 less
plete myotomy (single or multiple) of the inferior exotropia or more esotropia in upgaze. Bilateral
oblique in the inferior temporal quadrant, in my opin- superior oblique tenotomy for treating an A pattern
ion, is not effective. Some surgeons believe that this produces from 7 to 70 less exodeviation or more
surgery is an effective weakening procedure. esodeviation in downgaze. The average change of
Denervation with extirpation of the inferior oblique alignment in downgaze after bilateral superior
seems to be an unnecessarily complicated and exten- oblique tenotomy is approximately 30. With a fixed
sive way to treat a problem that already has a safe, amount of either inferior or superior oblique weaken-
simple, and effective solution although some prefer ing, more effect is produced in large A or V patterns
this procedure in cases of maximum inferior oblique and less effect in small A or V patterns. This effect
overaction. A modification of inferior oblique weak- represents a type of built-in safety factor for this sur-
ening is anterior transposition. This technique weak- gery. Bilateral superior oblique weakening proce-
ens the inferior oblique as much as myectomy or 8.0 dures produce approximately 6 of eso shift in the
mm recession. In addition, it seems to tether the eye primary position. Bilateral inferior oblique weaken-
and reduce the hyperdeviation or sursumduction in ing procedures produce little, if any, horizontal
patients with DVD. Anterior transposition of the change in the primary position.
inferior oblique is indicated in cases of DVD with
overaction of the inferior oblique and V pattern.
This procedure should only be done bilaterally. If
done in one eye, a restrictive hypotropia in upgaze is
produced causing a secondary deviation.
Inferior oblique strengthening:
Strengthening procedures of the inferior oblique,
in my experience, are seldom effective for treating
vertical misalignment; therefore, no figures can be
given for expected correction. If a tuck is performed,
no less than 10 mm of the muscle should be included.
If resection and advancement are undertaken, approx-
imately 5 mm of the muscle should be resected and
the muscle should be advanced 5 mm. I have done
this once or twice in nearly 40 years. Recession of
the yoke, contralateral superior rectus, is probably the
most effective treatment for the rare case of inferior The horizontal rectus muscles may be shifted
oblique palsy. one-half to one muscle width upward or downward.
This produces 10 to 15, or more change in larger
patterns of A or V. The medial rectus muscles are
always moved in the direction of the apex of the A
or V and the lateral rectus muscles are always
moved toward the open end of the A or V. Vertical
shift of the horizontal rectus muscles may be accom-
plished with symmetric surgery (bilateral recession or

115
Chapter 4

resections) or when a recession-resection is per- While patients with mechanical restriction and
formed. The horizontal rectus muscles also may be fusion potential are prime candidates for an
moved vertically without recession or resection in adjustable suture, fusion potential and diplopia are
cases of A or V pattern without oblique dysfunc- not absolute requirements. Cases not suitable for an
tion and when no horizontal deviation is present in adjustable suture are congenital esotropia, intermit-
the primary position. tent exotropia (some may disagree), dissociated verti-
Horizontal shift of the vertical rectus muscles cal deviation, any inferior oblique surgery, and sur-
can be done for treatment of A and V patterns. To gery for convergence insufficiency, etc.
decrease an esodeviation, the vertical recti are shifted An adjustable medial rectus recession may be
temporally and to decrease an exodeviation, the verti- combined with a muscle transfer procedure for treat-
cal recti are shifted nasally. For example, temporal ment of sixth nerve palsy. Some patients may be
shift of the inferior rectus muscles can be done in V adjusted at surgery on the table when local anesthet-
pattern esotropia in patients without a deviation in the ic is used, in the recovery room, or in the clinic on the
primary position and without oblique muscle dys- day following surgery. I have heard one very experi-
function. The best use of this information is when enced surgeon describe adjustment done more than a
recessing the inferior rectus in thyroid disease. Nasal week later! The alignment after adjustment may
shift of these muscles is done to avoid the postopera- remain stable or the alignment may shift over time
tive unwanted A pattern. postoperatively, but this can occur with any type of
strabismus surgery.
The faden operation (posterior Adjustable sutures are useful, but they are only a
fixation suture) small part of my surgical scheme. Some fine strabis-
mus surgeons use an adjustable suture in nearly every
The so-called faden operation should be called
case of rectus muscle surgery. Other surgeons never
posterior fixation suture or retroequatorial myopexy.
use an adjustable suture! Take your choice. When
This procedure has become a regular part of the sur-
used, I prefer the tandem adjustable suture (see page
gical armamentarium for many strabismus surgeons.
259).
It was performed initially for treating the nystagmus
blockage syndrome as suggested by Cppers. Peters Extraocular muscle transfer
described essentially the same procedure earlier, but
it did not catch on. Expanded use of the posterior fix- Extraocular muscle transfer procedures are indi-
ation suture includes any condition in which a sec- cated in complete or near-complete paralysis of a rec-
ondary deviation will promote comitance. This pro- tus muscle. Passive ductions must be free before the
cedure weakens a muscle in its field of action but has transfer is done if optimal results are expected. The
little effect, at least theoretically, on the primary posi- usual procedure in muscle transfer is to shift the
tion deviation or the action of the antagonist of the insertion of the two antagonist rectus muscles to a
operated muscle. In cases of esotropia with nystag- point at or near the insertion of the paretic rectus mus-
mus blockage, the posterior fixation suture is per- cle lying between them. The shift can be carried out
formed alone or is combined with appropriate reces- in some fashion to make up for a paretic medial rec-
sion of the medial rectus muscles. Results of this sur- tus, superior rectus, inferior rectus, or lateral rectus
gery are good, according to some, but when this pro- muscle. Superior oblique tendon transfer is more dif-
cedure is combined with a recession it is difficult to ficult to perform and frequently less effective. It is
know which part of the procedure affects the devia- usually done to reduce the exodeviation in third nerve
tion. palsy. Botox, injection of the antagonist of the paret-
ic muscle can be done in conjunction with a full ten-
Adjustable suture don transfer. Although rare, anterior segment
ischemia can occur when three rectus muscles are
I use an adjustable suture, when indicated, on
detached. Therefore, we like to avoid removing any
any of the rectus muscles and have attempted it with-
extra rectus muscles; Botox can help with this.
out success in a few cases involving the superior
oblique tendon. Indications for use of an adjustable Botox injection
suture are (1) restrictive strabismus in a patient with
fusion potential (the patient with thyroid ophthal- Botox injection remains a viable option for sev-
mopathy may be the prime example of such a eral categories of strabismus. We use it for some
patient); (2) any strabismus in which the outcome of cases of thyroid ophthalmopathy, persistent, residual,
surgery cannot be readily predicted (including or secondary strabismus, some small-angle devia-
patients who had previous unsuccessful surgery); and tions, and in cases where the patient does not wish to
(3) when this technique is expected to produce better have further incisional surgery. We do not use Botox
results according to the surgeons experience. for congenital-infantile esotropia or intermittent
exotropia. Alan Scott has suggested that Botox will

116
Workup of the strabismus patient

be useful for about 15% of strabismus patients. I use 3. The surgeon should aim at a cure with first
it in 3% of cases. A few surgeons use Botox for treat- surgery, provided there are sufficient muscles
ment of congenital esotropia and claim good results. to operate on without causing complications
John Lee of Moorfields in London has more experi- such as anterior segment ischemia.
ence than anyone I know of when it comes to use of 4. Because no procedure is ever 100% success-
Botox for strabismus and he employs it successfully ful, patients (parents) should be given a rea-
in a wide variety of cases. Emilio Campos has report- sonable estimate of the likelihood that a sec-
ed good results after treatment of congenital esotropia ond procedure will be required.
with Botox, but this method of treatment is not used 5. The surgeon should strive toward judicious
widely. boldness and not be excessively fearful of
producing an overcorrection.
Summary of steps 1 & 2 in the 6. If the surgeon is doing the proper amount of
design of strabismus surgery surgery, he or she should expect some over-
When an accurate workup has been completed corrections and should not produce an exces-
and a pertinent history recorded, the surgeon should sive number of undercorrections with regard
possess sufficient knowledge of the patient and the to intended results. For example, if a surgeon
strabismus problem to have certain treatment goals in aims at a 5 undercorrection, a patient who is
mind. In addition, the surgeon should also have real- ortho in the early postoperative period repre-
istic expectations of the results that could be expect- sents a relative overcorrection.
ed from surgery. That is, the surgeon should know 7. More effect is produced per millimeter of
approximately how much change in ocular alignment recession or resection by strabismus surgery
is expected with muscle strengthening, weakening, or in a child or in a patient with a small eye; less
transfer procedures appropriate for the patients stra- effect is produced by strabismus surgery in an
bismus. It is the union of these two factors, 1) patient adult or in a patient with a large eye.
findings and 2) results to be expected from surgery, 8. More effect is gained from strabismus sur-
that enables the surgeon to design each surgical pro- gery on a recent deviation than on a long-
cedure specifically for each patient. This combina- standing deviation.
tion of clinical findings and results expected is made 9. Surgery for a small deviation (25) pro-
more sensitive by the application of certain rules that duces less effect per millimeter of surgery
help the surgeon predict how certain types of patients than that for a large deviation (50).
may respond to strabismus surgery and how selected 10. In patients with cerebral palsy and strabis-
variables could affect the outcome. mus, the more cephalad the neurologic
It should be re-emphasized here that orthoptic, involvement, the longer surgery should be
optical, and to a lesser degree, pharmacologic therapy delayed. Patients with only limb involvement
can be an alternative and in selected cases a better in which the cranial nerves are spared may be
option than surgical therapy for strabismus in select- treated as otherwise normal strabismic chil-
ed patients provided these nonsurgical methods result dren.
in comfortable fusion and the accompanying accept- 11. In partially accommodative esotropia, only
able appearance. Surgery to restore ocular alignment the nonaccommodative part of the deviation
or to enable enhanced binocularity including fusion should be treated surgically.
should be reserved for patients who cannot be helped 12. One 4 mm medial rectus recession corrects
by other nonsurgical means alone. approximately 13 of esotropia.
13. Conjunctival recession is performed when
Step 3: Guidelines for conjunctival scarring causes restricted move-
ment or unsightly appearance.
application of surgical options 14. A minimal recession-resection for either
The third component in the design of the surgical esotropia or exotropia produces approximate-
procedure joins Step 1 (the workup) and Step 2 (sur- ly 25 reduction in the esodeviation or
gical options). The following aphorisms may be exodeviation.
applied to help produce a successful union between 15. A maximum recession-resection procedure
the patient and his or her surgical plan: for either esotropia or exotropia produces
1. If fusion is now or has ever been present, a approximately 50 reduction in the esodevia-
cure with fusion may be expected. A slight tion or exodeviation.
overcorrection may help obtain this result. 16. Three muscle surgery for esotropia or
2. If no fusion potential is present, a slight exotropia may be required for deviations
undercorrection is more likely to produce a between 50 and 75.
stable, small angle residual deviation. 17. Four muscle surgery for either esotropia or

117
Chapter 4

exotropia may be required for deviations rectus or with bilateral lateral rectus reces-
greater than 75 but rarely is performed in sion. Convergence insufficiency is treated
children. with bimedial rectus recession or recession-
18. Esodeviations or exodeviations greater than resection, in one eye.
50 in a patient with very poor vision in one 28. The timing of surgery for intermittent
eye should be treated with a supermaximal exotropia is dictated by how often the devia-
recession-resection of one eye to avoid sur- tion is manifested rather than the deviation
gery on the better eye. measurement.
19. Surgery for esotropia in a hyperkinetic child 29. Once surgery has been decided on for a
produces less effect than the same amount of patient with intermittent exotropia, the
surgery in a placid child. amount of surgery is dictated solely by the
20. Residual esotropia after a bimedial rectus angle of the deviation and is in no way influ-
recession that had been performed several enced by the amount of time deviation is
years before may be treated with re-recession either latent or manifest.
or a marginal myotomy of one previously 30. Bilateral inferior oblique myectomy produces
recessed medial rectus and a resection of one 20 less exotropia or more esotropia in
lateral rectus if the deviation is 25. This upgaze with no significant change of the
procedure should be bilateral if the deviation alignment in the primary position or in
is 50. For deviations between 30 and downgaze.
50, a resection of the lateral rectus alone 31. Unequal bilateral overaction of the inferior
may be performed in the second eye. obliques causing a V pattern should be treat-
21. Residual esotropia occurring weeks to ed with an equal weakening procedure on the
months after a bimedial rectus recession inferior obliques. If only the more overacting
should be treated with a bilateral lateral rectus inferior oblique is weakened, a markedly
resection or a re-recession of the already unequal overaction of the obliques with the
recessed medial rectus muscles. non-operated muscle becoming much more
22. A double 80% marginal myotomy combined overactive may occur postoperatively.
with a resection of the antagonist produces 32. Bilateral superior oblique tenotomy produces
the same weakening effect as a maximum a decrease in exotropia in downgaze between
recession of that muscle. A marginal myoto- 7 and 70. The average change is 30. The
my without resection of the antagonist is a smaller the A, the less the change in
less effective procedure. downgaze; the more the A, the greater the
23. Secondary exotropia after medial rectus change in downgaze.
recession that demonstrates deficient adduc- 33. Vertical shift of the horizontal rectus muscles
tion can be treated with medial rectus resec- for A and V patterns is accomplished as
tion and advancement. follows: the medial rectus muscles are shift-
24. A bilateral lateral rectus recession is per- ed toward the apex of the A or V; e.g. up
formed for exotropia less than 40 that is in A pattern and down in V pattern.
greater at distance with excess abduction and Lateral rectus muscles are moved toward the
equal vision. open end of the pattern; e.g. downward in A
25. Exotropic patients who have lateral incomi- pattern and upward in V pattern. Vertical
tance - that is, who have less exodeviation in shift of the horizontal rectus muscles (one-
lateral versions - tend to be overcorrected half to one muscle width) produces approxi-
more easily than patients whose exodeviation mately 10 to 15 change in the A and V
is the same in the primary position as in later- pattern. The greater the vertical incomitance,
al versions. the more the effect.
26. Exotropic patients who have had extensive 34. Horizontal shift of the vertical recti for treat-
preoperative orthoptics, especially near point ment of A and V is done by shifting the
of convergence exercises, are prone to large muscles temporally to treat esodeviation and
overcorrections after surgery. nasally to treat exodeviation.
27. The choice of muscles to be operated in the 35. Acquired non-traumatic, small angle superior
surgical treatment of intermittent exotropia is oblique palsy in an older patient should be
indicated by the pattern of deviation. evaluated according to the patient's needs.
Divergence excess exotropia is treated with This can be treated with prisms or patching
bilateral lateral rectus recession; basic for 4 to 6 months, and, if necessary, can be
exotropia and simulated divergence excess treated surgically. A medical workup should
exotropia are treated with a recession of the be done.
lateral rectus and a resection of the medial 36. Bilateral superior oblique palsy frequently

118
Workup of the strabismus patient

causes cyclotropia and cyclodiplopia that is 47. DVD with overaction of the inferior obliques
measured at 15 degrees or greater with the and V pattern is effectively treated with
double Maddox rod test. These patients typi- bilateral anterior transposition of the inferior
cally have a chin down, eyes up head posture obliques.
to obtain fusion or wear an eye patch. 48. Congenital superior oblique palsy frequently
37. When a large horizontal deviation and a small demonstrates no torsional response when test-
vertical deviation exist in a patient with no ing with the double Maddox rod test. In addi-
fusion potential only the horizontal deviation tion, spontaneous torsional diplopia is not
is treated surgically. observed. Acquired superior oblique palsy
38. A small vertical deviation in a patient with that is unilateral usually demonstrates torsion
diplopia and fusion potential may be treated with the double Maddox rod test but does not
with surgery and/or prisms. produce spontaneous torsional diplopia. The
39. An unacceptable vertical deviation with or torsion measured in unilateral superior
without fusion potential is treated surgically oblique palsy is less than 15. Both congeni-
by operating on the appropriate vertically act- tal and acquired superior oblique palsy are
ing muscles. The vertical rectus muscles frequently associated with an abnormal head
have more effect on the primary position posture. Bilateral superior oblique palsy
deviation than do the obliques. causes torsional diplopia and torsion greater
40. Brown's superior oblique tendon sheath syn- than 15 degrees with the double Maddox rod
drome is treated surgically only if a cosmeti- test. When greater than 15 degrees of tor-
cally unacceptable vertical strabismus or sional diplopia is found, the diagnosis is bilat-
abnormal head position is present while the eral superior oblique palsy unless ruled other-
patient is fixing in the primary position. wise. In addition, a spontaneous complaint of
41. Lysis of adhesions around an extraocular torsional diplopia is bilateral superior oblique
muscle is usually ineffective unless it is palsy unless ruled out.
accompanied by one or more of the following 49. The superior oblique muscle is the most com-
procedures: conjunctival recession, traction monly occurring anomalous extraocular mus-
suture placement, marginal myotomy, re- cle. If superior oblique palsy that is congeni-
recession, or re-resection. tal is also associated with amblyopia, hori-
42. Replacing tight or scarred conjunctiva to its zontal strabismus, anomalous structure or
preoperative position can nullify the results of even absence of the superior oblique tendon
otherwise potentially successful strabismus should be suspected.
surgery. 50. Superior oblique palsy with facial asymmetry
43. When there is a doubt about whether restrict- is likely to be congenital and to have an
ed motility could be caused by scarred con- anomalous tendon. The larger face is
junctiva, a conjunctival recession should be always on the side of the paretic superior
performed, leaving bare sclera. oblique. The smaller side of the face may
44. Long-term traction sutures should be be characterized by a smaller distance
anchored securely in the sclera or placed in between the lateral canthus and the corner of
the horizontal rectus insertions to avoid the mouth.
unnecessary contact with the cornea. They 51. Superior rectus recession has virtually no
should be tied over a bolster and left in place effect on the palpebral fissure; superior rectus
for several days and watched carefully with resection is likely to narrow the fissure.
the eye fixed in the duction opposite the 52. Inferior rectus recession can significantly
restriction several degrees past the midline. widen the palpebral fissure and inferior rectus
45. The functional improvement of straight eyes resection can narrow the palpebral fissure.
after surgery is compromised when red, 53. Inferior rectus recession is commonly associ-
unsightly scars remain in the conjunctiva. ated with slippage leading to excessive reces-
One should always attempt to retain a normal, sion, especially in thyroid eye disease.
white conjunctiva postoperatively. If this Each surgeon should add his or her own person-
cannot be accomplished otherwise, conjuncti- al guidelines for strabismus surgery to this list and
val recession and/or excision should be per- should delete from this list those aphorisms that do
formed. not apply to his or her experience.
46. Manifest DVD may be treated surgically by
recessing one or both superior rectus muscles
and if the DVD persists resection of one or
both inferior recti should be done.

119
Chapter 4

Step 4: Surgical technique


For successful strabismus surgery, the surgeon of occlusion amblyopia, this program which occludes
should adhere to the following guidelines: one eye at all times maintains a positive environment
1. Know the anatomy of all of the extraocular for development of best acuity by eliminating any
muscles and the surrounding fascial planes. need for suppression. Occlusion for treatment of
2. Carry out sharp dissection carefully. Avoid amblyopia is an important immediate antecedent of
blunt dissection. surgical therapy. Surgery is performed in most cases
3. Respect the conjunctiva, paying special atten- after free alternation has been established.
tion medially to the proper position of the Another scheme for amblyopia treatment is
caruncle and the plica semilunaris. occlusion of the sound eye during all of the waking
4. Observe strict hemostasis. hours combined with daily observation of the fixation
5. Keep tissue moist, but not flooded, with a pattern by the parents. Parents are instructed to begin
physiologic salt solution. alternate patching if they observe free alternation and
6. Use appropriate magnification and proper to continue this regimen until surgery. The important
illumination assists with visualization. points to remember are that occlusion amblyopia can
be produced quite rapidly in the infant and that sup-
Step 5: Follow-up of the pression does not occur if one eye is occluded. When
surgical patient patching is restricted to the preferred eye, careful
During the postoperative follow-up, the surgeon observation of the amblyopic infant should be main-
should: tained and alternate patching started when the child
1. Follow the patient postoperatively according to alternates fixation. This is continued until surgery is
a regular scheme and according to each performed. Some prefer alternate day patching even
patient's unique needs. For example, my rou- in alternating strabismus to avoid anomalous binocu-
tine for congenital esotropia is to see children lar relationships. According to this theory, it is better
1 week and 8 weeks postoperatively and then to perform surgery with a more or less clean slate.
as needed with at least two additional visits in This scheme has no beneficial effect on motor
the first year after surgery. Additional exami- response. A positive effect on sensory results has yet
nations are scheduled as needed. to be proven. Amblyopia treatment in the older child
2. Answer promptly (or have a physician familiar is not so intimately related to timing and techniques
with the case) any call about a patient during of surgery.
the first week to 10 days after eye muscle sur- In recent national collaborative studies carried
gery, as if you suspected an infection. Be sure out by the Pediatric Eye Disease Investigator Group
that office personnel are familiar with this pol- (PEDIG), shorter periods of occlusion and as an alter-
icy. native treatment atropine penalization have been
3. Watch for amblyopia. Check visual acuity by shown to be as effective as prescribed full-time occlu-
the best means possible at each postoperative sion. From these results it can be inferred that patch-
visit. Institute amblyopia therapy promptly, if ing prescribed as full time is often carried out less rig-
needed. orously than patients report. This has been corrobo-
4. Use prisms as needed, employing either rated by electronic patch time monitoring. These
Fresnel or permanent prism. PEDIG studies compared line of improvement, but
5. Use glasses or miotics as needed. did not require free alternation as the end point.
6. Do not procrastinate if reoperation is indicated.
Refraction
Treatment of amblyopia Refraction is carried out in infants under 1 year
The use of occlusion for treatment of amblyopia of age, 20 to 40 minutes after instillation of one or
in infancy before surgical treatment of congenital two drops of 0.5% cyclopentolate hydrochloride
esotropia can be quick and effective. After a few days (Cyclogyl) in each eye. After 1 year of age, 1%
or weeks of occlusion, amblyopia can be eliminated Cyclogyl is used. In heavily pigmented children, 1%
in favorable cases and alternate fixation achieved. On atropine solution is used by parents at home begin-
the other hand, improperly applied, overzealous ning 3 days before the examination with a total of
occlusion can produce amblyopia in the formerly pre- four drops being instilled in each eye. These parents
ferred eye of the very young child. von Noorden sug- are always warned of the signs of atropine systemic
gests an occlusion ratio of 2, 3, or 4 days of occlusion effects of flushing, fever, and hyperactive behavior.
for the sound eye and one day occlusion for the To avoid these problems, parents should use only one
amblyopic eye with the caveat that one eye is occlud- drop in each eye and should occlude the puncta for 30
ed at all times. In addition to reducing the likelihood seconds after the drops are given to avoid the child's

120
Workup of the strabismus patient

swallowing the medicine and experiencing systemic glasses; and (5) patients with high or relatively high
effect. It is also a good idea to put a drop in one eye hyperopic refractive error will eventually require
in the morning and in the second eye in the afternoon glasses.
to further reduce the chance of an unwanted systemic
effect. If it would be difficult for a patient to return Timing of surgical treatment
for a second visit for an atropine refraction, we add After deciding at what age the infant suspected
phenylephrine HCL 2.5% drops and repeat the of having strabismus is first seen and how amblyopia
Cyclogyl one or two times at 5- and 15- to 20-minute and refractive errors are to be managed, the timing of
intervals, respectively. However, we are satisfied that surgery must be considered. Unless one avoids see-
Cyclogyl is adequate to determine refractive errors in ing children early and/or opposes early surgery on
most infants and children. We do not use 2% theoretical grounds, any delay between completion of
Cyclogyl because of concern about dose-related toxi- nonsurgical treatment (treatment of amblyopia and
city. correction of hyperopia) and surgically straightening
the eyes must be defended. More surgeons are now
Spectacle prescription proceeding to early surgery for congenital esotropia.
Treatment of hyperopia with spectacles in the Surgical straightening of the eyes is done as soon as
esotropic infant can identify refractive esotropia in a amblyopia has been treated and the refractive-accom-
child as young as 6 months. Spectacles should be modative component has been ruled out as the cause
prescribed for all esotropic infants and children with of the esodeviation, in any patient aged six months
greater than +3.00 refractive error. In some cases, and older. Some surgeons, myself included, even
+3.00 glasses or even plus correction of lesser consider four months an appropriate age for surgery
strength is given if it is expected to reduce esotropia. to treat congenital esotropia.
Because this prescription is often not effective and the A factor contributing to my enthusiasm for early
child will need surgery anyway and may not wear surgery is the availability of competent pediatric
glasses afterward, a loaner glasses program can be anesthesia. Safe pediatric anesthesia includes posi-
instituted. Anticholinesterase drops in lieu of glasses tive airway control, use of agents that have a wide
can be used in these children. This medication can be margin of safety, and constant monitoring of heart
used in patients with residual esotropia, particularly if rate, oximetry, blood pressure, respiration, tempera-
a slight reduction in the angle will straighten the eyes ture, and expired CO2. In addition, a continuous
sufficiently to produce improved binocular function. intravenous drip ensures required hydration for the
A more difficult problem is the case of a young preoperatively starved child. Even more important,
child with straight eyes but high hyperopia. For the indwelling intravenous catheter drip allows a
example, we occasionally see a child of 2 or 3 years quick and reliable route for the emergency adminis-
of age with +4.00 hyperopia or greater and straight tration of medication. In the case of older chil-
eyes. No definitive treatment regimen is appropriate dren and adults, the timing of surgery depends for the
in all cases, but some guidelines can be established: most part on the wishes of the patient. When suffi-
(1) if any esodeviation latent or intermittent is detect- cient measurements have been obtained and the devi-
ed, glasses should be given or at least considered; (2) ation has stabilized (as in acute cranial nerve palsy),
if glasses are considered but not given, the parents surgery is offered to the patient and is scheduled at
should be instructed to watch for and report any the most convenient time.
esotropia and an early follow-up appointment should In adults and in cases of acquired strabismus,
be given; (3) if visual acuity is reduced in both eyes surgery is done when nonsurgical methods have
or if in the case of high hyperopia bilateral ametropic accomplished all that they can, the patients health
amblyopia is suspected, glasses should be given; permits, and when the patient wishes.
(4) the higher the hyperopia, the greater the need for

121
5
Diagnostic categories and
classifications of strabismus

This chapter offers a comprehensive classifica- satory head posture. For example, those with con-
tion of strabismus and then provides a detailed genital third nerve palsy, in most cases, never had
description of the more common strabismus types normal fusion, but there is no reason to believe this is
including treatment options. My premise for what why the strabismus is present. On the contrary, motor
could be an unorthodox way to classify strabismus is fusion never developed or was lost because the eyes
that there are only two kinds of strabismus. These are were constantly misaligned because of the cranial
congenital esotropia and its sequelae and all the rest! nerve palsy. Individuals with intermittent exotropia
These two classes of strabismus are divided accord- have excellent fusion interspersed with periods of
ing to the presence or absence of an inborn motor deep suppression. Patients with Brown syndrome or
fusion mechanism (Figure 1). Duane syndrome can have a severe motor dysfunc-
Claud Worth said that congenital esotropia tion that the patient responds to by assuming an
results from a defect in the fusion faculty. Whether anomalous head posture, allowing normal binocular
this so-called fusion faculty refers to sensory fusion vision - fusion. The reason for including in all the
or motor fusion is not agreed upon by experts. rest acquired strabismus such as cranial nerve palsy,
Sensory fusion is the simultaneous perception of refractive esotropia, and mechanical causes such as
slightly different images from the two eyes blending blowout fracture, etc, is obvious.
them into a single object. This object is seen in depth. This more or less arbitrary method of dividing
Motor fusion is the alignment of the visual axes by strabismus has some value in that it helps predict out-
action of the muscles so that an object is seen as one come while guiding treatment. For example, congen-
rather than doubled. This image seen as one by virtue ital esotropia patients are not expected to have normal
of motor fusion may be seen in depth as a result of fusion, regardless of timing and type of treatment,
sensory fusion or may not, at least with clinical tests and are subject to a variety of other strabismus con-
such as the Polaroid vectograph test used in the clini- ditions, most notable of which is dissociated strabis-
cal setting. mus.* In contrast, in the all the rest category many
All the rest of the strabismus entities consist of other types of strabismus, depending on severity and
those who have strabismus, but were born with, or duration, retain the potential for normal or a near nor-
presumably born with the potential for bifoveal mal sensory result after timely and effective treat-
fusion. In this category of strabismus, fusion is lost ment.
from a variety of causes other than a primary inborn Later in Chapter 15 and 16, a wide variety of
deficiency in the central motor fusion mechanism. examples of strabismus will be presented describing
The individuals in the strabismus category, all the clinical characteristics, treatment and results. These
rest, can be said to have either had the potential for will include the more or less naturally occurring types
fusion, but it was lost never to be regained; demon- as well as strabismus from trauma and after prior sur-
strate fusion only part of the time; or appreciate gical treatment.
fusion full time, or nearly so, by assuming a compen-

* Those cases of congenital esotropia who are said to have normal fusion do not, in my opinion, have by definition congenital esotropia.

123
Chapter 5

Figure 1 A comprehensive classification of strabismus

124
Diagnostic categories & classification of strabismus

Congenital esotropia
The most common form of strabismus is an about the time of onset. The term essential has been
esodeviation with onset during infancy occurring in added to make it clear that we do not know the cause
an otherwise neurologically normal infant. It is of this esodeviation. A paucity of information about
labeled congenital, infantile, or essential infantile ocular motility in the normal newborn as well as in
esotropia. These patients can have other physical the newborn and infant with early onset esodeviation
signs including face turn and nystagmus. Although left strabismologists with several descriptive terms,
the clinical characteristics of this strabismus entity but little understanding of mechanisms.
have been thoroughly described, many questions In the past ten years, new information has
remain about: timing of onset, etiology, terminology, accrued from study of ocular motility in the normal
and treatment outcome. This is understandable newborn. In addition, a national collaborative study
because, for the most part, the earliest stages of this of the clinical behavior of esotropic infants beginning
strabismus have been recorded mostly by parents or at just a few weeks of life has determined that the
pediatricians. These esotropic infants had not been diagnosis of congenital esotropia can be made with
subjected to careful study in large numbers in the confidence at 4 months. This study also suggests to
past by the ophthalmologist or the basic scientist me that surgery can be done as early as four months
studying infant psychophysics. of age to treat a constant esodeviation. Supported by
Now this is changing. The issues surrounding this type of information, surgery is now being done
the causes and early findings in congenital esotropia on infants as young as four months and in some cases,
are being discussed in a new light. The competing younger. Data generated from this experience has
ideas that congenital esotropia is a primary defect of also provided information about the best treatment
the fusion faculty (Worth) or that this condition results that can be obtained. Now based on my own
develops as the otherwise normal binocular system clinical experience and on interpretation of available
is overcome by esotropital factors causing a sec- clinical and laboratory data, I will present my view of
ondary esotropia (Chavasse) are being subjected to the broad clinical picture or what I will call congeni-
scrutiny in the laboratory and the clinic. tal esotropia.
Whether the so-called fusion faculty of Worth is
related to sensory fusion or motor fusion has never Terminology
been clearly stated, but careful reading of Worth indi- The term congenital esotropia was popular-
cates, at least to me, that he was referring to motor ized by Costenbader. However, the word congenital
fusion. This suggests that adequate treatment of con- has been challenged because this condition is not con-
genital esotropia in the form of surgical realignment firmed at birth, except, in some cases, by parents. In
of the eye can improve alignment leading to excellent other words, the esotropia is not conatal, at least as
appearance and peripheral fusion or subnormal binoc- confirmed by expert observation. To counter this
ular vision but treatment cannot achieve normal objection it could be pointed out that other conditions
motor or what depends on normal motor fusion, nor- termed congenital are not necessarily conatal. For
mal sensory function. example, so-called congenital subluxation of the hip
The theory of Chavasse suggesting that a time- is not manifest in most cases until several months
ly reversal of esotropital factors could result in nor- after birth. However, it is arguable to label something
mal fusion although not in my opinion correct, was as congenital if the hidden precursor of a later mani-
the impetus for earlier surgery for congenital festing condition is said to be present at birth. This
esotropia. This was championed by Frank may be the case with congenital esotropia. As an
Costenbader and later Marshall Parks. Their efforts alternative, the term infantile esotropia has been
supported by improved surgical anesthesia, finer used because it more accurately describes the time of
suture, sharper-finer needles, better instruments, and onset of the esodeviation, that is, during infancy, beg-
effective magnification, and carried on by a cadre of ging the question When did the underlying cause
well-trained young strabismologists has resulted in originate? Discouraging use of infantile esotropia,
many ophthalmologists treating congenital esotropia Lang and Parks defend the term congenital
successfully with surgery at 6 months and even esotropia. Parks stressed that it is an established
younger. Some have even claimed near perfect motor term describing an entity whose clinical characteris-
and sensory results in a few cases. These cases tics and response to treatment are well known to all
notwithstanding, the best results from treatment of strabismologists.
congenital esotropia seems to be better but not per- Supporting use of the term infantile esotropia,
fect and then only with close postoperative follow up von Noorden said, I prefer...infantile esotropia to
and appropriate intervention. describe a constant deviation with a documented
The terms congenital and infantile have been onset during the first six months of life and add the
vigorously debated mostly because of uncertainty prefix essential to emphasize the unknown origin

125
Chapter 5

and to distinguish it from other forms of esotropia In addition, it must be noted that the infant has either
that occur at birth or during infancy. While I could alternation, often with cross fixation or fixation pref-
defend the term infantile esotropia, I am convinced erence for one eye, implying amblyopia. Other com-
that affected infants have an inborn defect in motor mon clinical findings often present but not essential
fusion. to the diagnosis are: manifest nystagmus, oblique
Is this controversy regarding terminology muscle dysfunction, dissociated strabismus either
important? vertical deviation (DVD) or a horizontal deviation,
primarily an exodeviation of one eye (DHD), variable
The crux of the argument regarding the validity
angle, latent nystagmus, manifest latent nystagmus,
of the terms congenital versus infantile may
and torticollis.
depend on when the initial defect leading to the stra-
All patients with congenital esotropia have
bismus occurs, rather than the timing of the manifest
compromised sensory functioning because the object
esotropia itself. The question is: Does the infant
of regard is seen by the fovea of one eye and nasal
who ultimately is diagnosed as congenital or essen-
retina of the other eye. Stated another way, anatomi-
tial infantile or simply infantile esotropia start life
cally corresponding parts of the visual system will
with the potential for normal binocular vision and
always be seeing something different. This is the
lose it because of acquired motor induced factors, or
basis for development of anomalous retinal corre-
does the infant begin life with an inborn lack of nor-
spondence, which in my opinion is always harmo-
mal binocularity because of a central defect that even-
nious (equal to the objective angle of strabismus) if
tually causes an esodeviation by a failure to provide a
tested with a minimally dissociating stimulus. Non-
template in the central nervous system on which the
harmonious anomalous retinal correspondence, in my
normal binocular motor fusion develops? This may
opinion, is a testing artifact, but this finding may have
be nothing more than a restatement of the arguments
some use as a measure of the depth of adaptation.
surrounding the Worth (fusion faculty) versus
Late sequelae of congenital esotropia with and
Chavasse (motor reflexogenic) theories for the ori-
without treatment include some or all of the follow-
gins of infantile esotropia.
ing: secondary exotropia (large angle, late occur-
Whether the esodeviation is called congenital
ring), DVD, DHD, amblyopia, overaction of the
or infantile may be considered unimportant provided
inferior obliques with V pattern, overaction of the
the etiology remains unknown. On the other hand,
superior obliques with A pattern, X pattern with the
the design of more effective treatment may depend on
overaction of all obliques, and recurrent esodeviation
better understanding of etiology. von Noorden stated,
with or without the influence of a refractive or accom-
If etiology is implied, terminology is important,
modative component. Individuals with congenital
and I tend to agree. Hereafter in this section, using
esotropia have asymmetric optokinetic nystagmus
von Noordens argument but coming to a different
characterized by a normal nystagmus beat for nasally
conclusion, I will use the term congenital esotropia.
directed targets and an abnormal response to tempo-
Characteristics rally directed targets both before and after treatment.
Ciancia has also demonstrated a preponderance of
According to results from the Pediatric Eye response from crossing (nasal retinal) optic nerve
Disease Investigator Group (PEDIG), congenital fibers when comparing hemispheric visually evoked
(infantile) esotropia can be confirmed by a reliable potential (VEP) response in congenital esotropia.
observer by 4 months of age. The minimum required I have examined several patients with most of
findings for diagnosis are as follows:* the typical characteristics of congenital esotropia, but
1. Esotropia--usually 40 to 50 diopters, but with no esotropia! One patient was seen initially
with a range of 10 to 90 prism diopters. under a year of age with DVD but normally aligned
2. Normal neurologic status (except for stra- eyes. She was followed until age 7 years at which
bismus) time accurate sensory testing could be completed.
3. Refractive error expected for age (usually This patient at this time had DVD, asymmetric OKN
low to moderate hyperopia), correction of refractive esotropia and only gross stereo acuity.
which does not eliminate esotropia. Another, a teenage boy, the sibling of a congenital
4. Asymmetric optokinetic nystagmus charac- esotropia patient, had DVD and OKN asymmetry, but
terized by robust temporal to nasal response his eyes were aligned and he demonstrated normal
and erratic nasal to temporal response. stereopsis! Patients with these characteristics have

* An infant with 40 prism diopters or more of constant esotropia at 4 months has a 100% chance of having esotropia at 7 months. If at
4 months the esotropia is less than 40 prism diopters or is intermittent, the likelihood of there being a constant esotropia at 7 months is
70%.
It is unlikely that true overaction of the oblique muscles exists. Instead this could be over expression because of a weak antagonist
or deficient adduction allowing increased expression of the secondary abducting action of the oblique muscles.

126
Diagnostic categories & classification of strabismus

been diagnosed as having congenital esotropia sine


(without) strabismus. This suggests that congenital Subnormal binocular vision*
esotropia exists on a continuum and is genetically
influenced. Orthotropia or heterophoria
Normal visual acuity in both eyes
Treatment Fusional amplitudes
Normal retinal correspondence
While terminology and theories of origin may
Foveal suppression in one eye in binocular vision
be debated, it is the nearly universal belief of strabis-
Reduced or absent stereopsis
mologists that surgery is the treatment of choice after
Stability of alignment
refractive/accommodative components have either
Optimal treatment result for congenital esotropia
been ruled out or treated and after any amblyopia (if
present) has been treated. Ideally these patients *We have examined normal parents of children with congen-
ital esotropia and found that they have reduced stereo acuity
should alternate fixation before surgery, but surgery is indicating subnormal binocular vision.
often carried out before this is accomplished.
Amblyopia therapy in such cases can be continued
Microtropia
after surgery. The age chosen for surgery depends on
the surgeons preference and varies from as early as 4 Inconspicuous shift or no shift on cover test
months to 3 years of age or more. The trend, howev- Mild amblyopia frequent
er, is for surgery to be done at a younger age. Fixation central or parafoveolar in one eye
Chemodenervation of both medial recti with Botox Fusional amplitudes
has been done, but has not achieved a prominent role. Anomalous retinal correspondence (if small shift
(see chapter 14). on cover test)
Surgery for infantile esotropia consists of bime- Reduced or absent stereopsis
dial rectus recession, medial rectus resection, and lat- Fairly stable alignment
eral rectus resection of one eye, a three-muscle pro- No further treatment except amblyopia preven-
cedure combining a bimedial rectus recession with a tion
resection of one lateral rectus or in a very few cases a Desirable treatment result
four-muscle procedure consisting of a bilateral reces- Microtropia with identity has harmonious anomalous corre-
sion-resection. In cases with inferior oblique overac- spondence between the eccentric fixation of the amblyopic
tion and V pattern, both inferior oblique muscles may eye and the fovea of the sound eye.
also be weakened at the initial surgical procedure.
These patients often have lax superior oblique ten- Monofixation (Parks)
dons that could be treated either with bilateral superi-
or oblique tuck or bilateral inferior oblique weaken- Esotropia (XT) < 10 prism diopters
ing. Most cases of infantile esotropia, particularly HARC
those done at less than 1 year, have only horizontal Alternation or amblyopia
rectus muscle surgery done at the initial procedure. Fusional amplitudes
Inferior oblique overaction may occur months to Stable angle
years after initial horizontal muscle surgery in which Good result
case inferior oblique weakening is done as a second According to Parks peripheral NRC.
procedure.
The best attainable result from treatment for Small-angle esotropia/exotropia
congenital esotropia in my experience is subnormal (<20 prism diopters)
binocular vision (Table 1). However, Kenneth W.
Wright has reported a patient aligned at 2 months and Cosmetically acceptable?
28 days who obtained alignment and stereo acuity of 80% have anomalous retinal correspondence
40 seconds arc disparity. An acceptable but less desir- Less stability of angle
able result is microtropia, and a still less desirable Further surgery based on appearance; amblyopia
result is small-angle eso- or exotropia. Large-angle treatment as needed; may benefit from correction
eso- or exotropia, a still less desirable result, requires of hyperopia
further surgery. We have found that 80%+ of patients May be acceptable result
treated surgically in our clinic for congenital I believe that all strabismus patients have harmonious anom-
esotropia have good initial results; that is, small-angle alous retinal correspondence if tested with tests which disas-
esotropia or exotropia of less than 10 prism diopters, sociate eyes less such as Bagolini glasses.
microtropia, or subnormal binocular vision. With
continuing treatment nearly 100% of congenital
Table 1 Results of treatment
esotropia patients are aligned in the primary position
by their teen years. von Noorden confirmed the ear-

127
Chapter 5

given a second chance by having the eyes straight-


Large-angle esotropia/exotropia ened early. Of the two, I believe the former alterna-
(>20 prism diopters) tive to be the most likely.
Usually cosmetically unacceptable
Less chance for anomalous retinal Results of early surgery for congenital
correspondence, suppression prevails esotropia
Unstable angle In an effort to learn more about the results that
Unacceptable result can be expected from early surgery for congenital
Further surgery indicated esotropia we collected ten patients who underwent
bimedial rectus recession between 83 and 159 days of
HARC can be found.
From von Noorden GK: A reassessment of infantile age. The preoperative characteristics are shown
esotropia, XLIV Edward Jackson Memorial Lecture, Am J (Table 2). These patients were examined initially
Ophthalmol 105:1-10, 1988 between 55 and 150 days. They had an average devi-
ation of 50 prism diopters with a range of 35 to 60
Table 1, contd Results of treatment
prism diopters. Most were noted by their parents to
have crossed eyes at birth. The refractive error
ranged from +1.00 to +3.00 diopters. Three patients
lier findings of Ing, Parks, and Costenbader that sur- demonstrated fixation preference suggesting the pres-
gery done before 2 years of age produces better sen- ence of amblyopia.
sory results compared with surgery done between 2 Initial surgery was bimedial rectus recession
and 4 years of age and surgery done after 4 years of between 8.5 and 10.0 mm measured from the limbus.
age. In my experience, the best result from surgery In each case the eyes were aligned in the immediate
for congenital esotropia falls short of normal binocu- postoperative period (Table 3). These patients were
lar vision with perfect stereo acuity. That is, eyes followed, for a period of 8.3 years (the least) to 11.8
may be aligned with equal vision but stereo vision is years (the longest). They were seen collectively a
not normal. Anecdotal reports of infantile esotropia total of 221 times in follow up.
treatment resulting in normal stereopsis of 40 seconds Only three of the ten patients remained aligned
of arc disparity beg the question, Was this infantile with a single surgery. The other 7 required a total of
esotropia? I believe these patients could represent 11 additional surgical procedures. Three had just one
either early acquired esotropia in a child born with additional surgery. Three needed two additional sur-
normal sensory potential or a rescue of sensory geries and one patient required three additional surgi-
function in an infant who could capitalize on being cal procedures (Table 4).

Table 2 Preoperative Patient Characteristics


From Helveston EM, et al. Results of early alignment of congenital esotropia. Ophthalmology, 1999, 106(9):1716-1726.
Used with permission.

128
Diagnostic categories & classification of strabismus

Table 3 Initial Surgery


From Helveston EM, et al. Results of early alignment of congenital esotropia. Ophthalmology, 1999, 106(9):1716-1726.
Used with permission.

Table 4 Secondary Surgeries


From Helveston EM, et al. Results of early alignment of congenital esotropia. Ophthalmology, 1999, 106(9):1716-1726.
Used with permission.

During the course of follow up, two patients to temporal. This response seems to be a common
required hyperopic correction to maintain alignment* denominator of congenital esotropia. That is, all con-
and two patients required a short period of occlusion genital esotropia patients demonstrate this and con-
for amblyopia. At the end of the follow up period 19 versely if a patient demonstrates asymmetric OKN
of 20 eyes had visual acuity of 20/40 or better. All response, he/she has congenital esotropia.
patients were aligned to within 10 prism diopters of Looking at the results of surgical treatment for
orthotropia at distance, near, or both. Nine of 10 congenital esotropia reported by seven investigators,
patients had dissociated vertical deviation and 4 had the following becomes clear: (Table 6)
latent nystagmus. Four patients had measurable 1. Stereo acuity is attainable, but it is reduced.
stereo acuity, two at 3,000 seconds (stereo fly), one at 2. Most patients require at least one additional
400 seconds, and one at 140 seconds (Table 5). surgery.
All of the patients demonstrated optokinetic 3. Dissociated vertical deviation occurs in most.
asymmetry which is characterized by smooth pursuit It has been suggested that early surgery in the
of stripes moving from temporal to nasal and jerky patient with congenital esotropia might lead to a high-
eye movement response to stripes moving from nasal er incidence of dissociated vertical deviation. To test

* Both had less than +3.00 refraction before surgery. The patient who was +3.00 before surgery was plano at the last visit.

129
Chapter 5

Table 5 Long-term outcome of early surgery for congenital esotropia


From Helveston EM, et al. Results of early alignment of congenital esotropia. Ophthalmology, 1999, 106(9):1716-1726.
Used with permission.

Table 6 Summary of results of treatment of congenital esotropia


From Helveston EM, et al. Results of early alignment of congenital esotropia. Ophthalmology, 1999, 106(9):1716-1726.
Used with permission.

this, Neely, et. al. in a retrospective study demon- The dynamic nature of congenital esotropia is
strated that DVD developed approximately 18 demonstrated by the logs of four patients in this study.
months after surgical alignment in congenital A record of each visit along with preoperative pic-
esotropia patients aligned both early and at a later tures and a picture at the end of follow up show the
time. This study concluded that the timing of surgery satisfactory cosmetic results obtained in these
for congenital esotropia had no bearing on the occur- patients after a total of 97 visits and 17 interventions
rence of DVD. Rather, DVD occurs about one and a (including surgery, patching, and prescription of
half years after surgery for esotropia, regardless of the glasses). (Tables 7-10)
surgery being done early or later.

130
Diagnostic categories & classification of strabismus

Table 7 Case #5
From Helveston EM, et al. Results of early alignment of congenital esotropia. Ophthalmology, 1999, 106(9):1716-1726.
Used with permission.

Table 8 Case #1
From Helveston EM, et al. Results of early alignment of congenital esotropia. Ophthalmology, 1999, 106(9):1716-1726.
Used with permission.

131
Chapter 5

Table 9 Case #7
From Helveston EM, et al. Results of early alignment of congenital esotropia. Ophthalmology, 1999, 106(9):1716-1726.
Used with permission.

Table 10 Case #10


From Helveston EM, et al. Results of early alignment of congenital esotropia. Ophthalmology, 1999, 106(9):1716-1726.
Used with permission.

132
Diagnostic categories & classification of strabismus

done in the newborn nursery study. None of the new-


Based on these findings and other clinical borns examined were found to have what could be
experience when discussing treatment options called typical congenital or essential infantile
with the parent(s) of a patient with congenital esotropia. Freidrich and deDecker later did a compa-
esotropia, I say the following: rable evaluation in 1000 newborns with similar
1. A diagnosis of congenital esotropia can be results.
made with confidence by 4 months of age During periods of observation, infants were frequent-
if the deviation is 40 prism diopters or ly noted to have both eyes crossed or both eyes
more, is constant, and no other neurologic diverged. This behavior was called convergence or
problems exist. divergence and not esotropia or exotropia.
2. Surgery can be done safely in an infant at Strabismus was considered to be present only when at
age 4 months if competent pediatric anes- least one eye fixed on the examiners face.
thesia is available. The conclusions from these newborn motility studies
3. An average of 2 surgical procedures is were as follows:
required to achieve stable alignment. 1. The clinical picture of congenital or infan-
Some patients are aligned with only one tile esotropia is not present at birth.
procedure, but others require more, even 2. Newborns seem to have an immature oculo-
up to a total of 4 (possibly more?). motor system with inaccurate, unsteady, and
4. For best results, children treated for con- nonmaintained fixation and they display a
genital esotropia should be followed tendency toward exodeviation.
closely until a time near the teen years. 3. Newborns are mainly visually inattentive dur-
5. Normal binocular function is not obtained ing short periods of observation and fix on the
from treatment of congenital esotropia, examiners face only for brief intervals.
but about one half of children can appre-
ciate some level of stereo acuity.
6. It has been my experience that alignment
achieved by the teen years in patients
treated for congenital esotropia tends to be
stable.

Ocular motility studies in the newborn


In an effort to learn more about the origins of
infantile esotropia, I examined 500 newborns in the
nursery as a pilot study to determine the status of ocu-
lar motility during the first few hours of life. Most of
the newborns I observed either had straight eyes or
were exodeviated. None had a picture compatible Figure 2
with as diagnosis or even a suspicion of congenital- Examination of motility in a newborn.
infantile esotropia. The study was continued with
Robert Nixon as the principal nursery examiner,
examining 1219 newborns (Figure 2). The alignment
pattern in these infants was as follows: 48.6% were
orthotropic, 32.7% were exotropic, 3.2% were exo- to
esodeviated, and 15.4% were uncooperative and no
statement was made about their motility.
In the third stage of the study, Archer, Sondhi,
Helveston, and Miller examined 2917 newborns and
found exodeviation in 66.5%, orthotropia in 29.9%,
and eso- to exodeviation in 3.6% (Figure 3). With
persistence and by using improved techniques carried
out principally by Archer, nearly all newborns were
sufficiently alert and cooperative to be examined dur-
ing the third phase of the nursery study. The higher
percentage of exodeviation is attributed to a longer,
more thorough period of observation in the nursery. Figure 3
Follow-up motility evaluation in newborn at a clinic.
In total, more than 4000 infant examinations were

133
Chapter 5

Follow-up evaluation of motility:


The orthotropization curve
A follow-up study of motility was also conducted on described as an orthotropization curve (Figure 4).
this newborn population as they returned to the well During this study, the Bruckner test was done by
baby clinic. In the entire study, which was led by the observing the binocular pupillary reflexes indicating
efforts of Archer, a total of 6228 examinations were bifoveal fusion in the infants followed during this
performed on 4211 infants, of whom 3324 otherwise study. The Bruckner test is done by observing the
normal infants formed the population base. Of these, binocular pupillary reflex with a plus lens in the
922 were seen an average of 1.5 times in addition to direct ophthalmoscope. Equal pupillary reflexes indi-
the newborn examination and 582 had their last cate bifoveal fixation and therefore alignment of the
examination after 4 months. The purpose of this visual axes. Unequal reflexes indicate central fixa-
study was to determine the prevalence of various tion with one eye and misalignment of the other eye.
motility findings during this period of observation. Unequal pupillary reflexes are also seen with straight
The results showed that esodeviations were not seen eyes when anisometropia is present.
in otherwise normal children after 2 or 3 months of Three children seen in the newborn nursery and noted
age. Exodeviations disappeared by 6 to 8 months of to have eyes that were straight or exotropic were
age. The alignment between birth and 1 month was found by age 6 months to have findings typical of
essentially the same. These overall findings were congenital esotropia (Figure 5). Three patients with
neonatal unilateral sixth nerve palsy reverted to nor-
mal within weeks to months. Another patient identi-
fied with Duane syndrome in the newborn nursery
and followed for several months continued to mani-
fest a typical type I esotropic Duane syndrome.
Conclusions from these observations of newborn
were the following:
1. The clinical picture of congenital-infantile
esotropia was not present at birth in 4,211
infants.
2. The majority of newborns have an unstable
ocular alignment biased toward an exodevia-
tion, and this persists for the first few weeks
of life.
3. Alignment at birth and at 1 month is similar.
4. There is a steady progression toward longer
periods of straight eyes during the first six
months of life.
5. Esodeviation persisting after two months of
age is abnormal motility.
6. Normal infants do not seem to have a period
of normal binocular stimulation prior to a pre-
sumed esotropital event such as early onset
refractive esotropia or any presumed periph-
eral motor--induced esotropia.
7. Normal infants as well as infants who are des-
tined to develop congenital esotropia may be
indistinguishable in the first two months by
clinic means.
8. Prolonged esotropia in the first weeks or
months does not preclude normal binoculari-
ty later.
Figure 4 9. Exodeviation persisting beyond six to eight
Orthotropization curve months is abnormal motility.
10. Duane can be diagnosed in the neonatal peri-
od.

134
Diagnostic categories & classification of strabismus

mal infant, but asymmetry persists in infants who


have congenital esotropia. It is not clear whether this
is a purely sensory or combined sensory-motor
response. It has been postulated that this response is
mediated through the brain stem at the nucleus of the
optic tract (NOT) based on anomalous cortical-retinal
interaction to visual stimuli. Ciancia has shown what
may be a purely sensory side of this phenomenon of
OKN asymmetry by demonstrating asymmetric visu-
ally evoked potential in this type of patient.
Between two and four months of age a stereo
response can be obtained for the first time using
either stable targets or the dynamic random dot stere-
ogram in infants with straight eyes. This stereo
response is never found in an esotropic individual or
in an infant less than two months old. According to
Leguire and to Archer, in separate studies, a few
infants who had their eyes surgically straightened at
an early age demonstrated gross stereopsis while the
eyes were perfectly straight but lost stereopsis as a
small-angle esodeviation returned.
For approximately the first four months the
infants visual-sensory-fusion system and visual-
motor systems are immature and relatively ineffective
Figure 5 compared to adult standards of acuity and alignment.
A A 1-day-old infant demonstrated slightly exodeviated The system in the normal infant will not sustain
eyes during examination straight eyes consistently until somewhere between
B At 6 months of age, this same child had 40 prism
two and four months of age.
diopters of esotropia and presented the typical findings
of congenital esotropia.
What causes congenital esotropia?
A theory for the origin of congenital esotropia
is proposed as follows:
The newborn visual sensory system 1. At birth infants who are otherwise normal
It is well known that visual acuity of the infant neurologically--and regardless of their ulti-
is significantly below the level found in adults. This mate ocular alignment--start with similar
visual acuity improves rapidly over the first few motor and sensory behavior. They have
weeks to months of life in the human, approaching reduced visual acuity (sensory), unsteady
adult visual acuity levels between six months and one ocular alignment (motor), and absence of
year of age. Psychophysical studies using forced stereopsis (sensory fusion) (Figure 6A).
preferential looking techniques have shown that dur- (Infants with obvious afferent defects such
ing the first month of life, infants prefer to look at as cataracts, anatomic defects such as optic
stripes of 18 cy/degree, improving to a 6 cy/degree by nerve hypoplasia, structural muscle or nerve
six months. This is compared roughly to acuity of defects, congenital motor nystagmus, sixth
20/800 and 20/100 in Snellen equivalents. However, nerve palsy Mbius, Duane syndrome, etc.,
this recognition acuity using high-contrast stripes is are considered separately).
not the same as resolution acuity measured with 2. The sensory and motor systems mature rap-
Snellen optotypes, the latter being a more exacting idly, concurrently, but independently. At
measure. It has also been shown histologically that approximately two months of age they are
the macula is immature at birth, approaching a mature sufficiently developed to sustain normal
appearance in the second year and not developing full binocular vision (Figure 6B).
photoreceptor maturation until the fourth year. 3. When the sensory and motor functions are
Newborns studied in the nursery support this finding sufficiently mature, they interact, through the
by their fixation behavior. It appears, however, that meditation of an inborn cortical fusion facul-
accurate central fixation with visual acuity approach- ty that has both a motor fusion and a sensory
ing 20/30 to 20/40 precedes histologic maturation. fusion component. This is the keystone of
Asymmetric optokinetic response is common in the arch that has a sensory arm and a
infants in the first three months of life. This response motor arm.
becomes symmetrical after three months in the nor-

135
Chapter 5

A B

Figure 6
A Before 2 months of age, the motor and sensory systems B Completed arch between 2 and 4 months.
are immature.

4. The motor fusion component of the key-


stone uses images disparity as a stimulus for
the acquisition of a single superimposed image
from each of the two eyes. The sensory com-
ponent of fusion recognizes small lateral dis-
placement of these two images (within Panums
fusional space) and interprets them as a single
image with stereoscopic depth perception.
Motor fusion can be normal in the absence of
sensory fusion, but sensory fusion is never nor-
mal in the absence of normal motor fusion
(Figure 7).
5. The fusion arch completes a stable union of
Figure 7
sensory and motor function maintaining The completed arch, crowned with stereopsis.
straight eyes.
If the keystone of motor-sensory fusion is
absent or defective, the developing system breaks
down and the arch collapses (Figure 8). The integri-
ty is lost because of a lack of motor fusion (absence
of the normal keystone of the fusion arch).
Strabismus occurs, and persists, in the absence of the
motor fusion lock in the infant, usually beginning
with a variable angle of strabismus and later devel-
oping into a stable angle. A secondary defect in the
afferent system (amblyopia) may occur because the
misaligned visual axes force one eye to be chosen to
view the object of regard and in the process of retinal
rivalry a stimulus for suppression occurs. Anomalous
retinal correspondence (ARC) then occurs as an adap-
tive mechanism in response to misalignment of the
visual axes. In my opinion, ARC is present in every
case of misalignment of the visual axes without Figure 8
diplopia, but it may or may not be found clinically, or The absence of the keystone of sensorimotor fusion caus-
may be found as non-harmonious ARC, depending on es a collapse of the forming arch of normal binocular vision
the method of testing. resulting here in esotropia.
Other reasons for collapse in the arch can be
defective sensory limb (congenital cataract) or motor
abnormalities (congenital third nerve palsy) produc-
ing manifest strabismus during the period of suscep-
tibility (Figure 9).

136
Diagnostic categories & classification of strabismus

Figure 9: The arch of normal binocular vision can be


destroyed in the sensitive period by failure of the motor side
or the sensory side.

Infantile esotropia with nystagmus as a promi- esotropia. In spite of having at least 20/20 vision in
nent feature may be an example of a combined motor each eye and no more than 2 prism diopters of phoria,
and brain stem--derived esotropia. Nystagmus block- these parents had a 16% incidence of reduced stereo
age type esotropia with manifest latent or manifest acuity of 80 sec arc or less and could be classified as
nystagmus may represent a secondary defect having subnormal binocular vision. This was in con-
(esotropia) occurring after convergence to damp nys- trast to a control group of parents who had a 2% inci-
tagmus. Those cases of congenital esotropia without dence of subnormal binocular vision. Adding to these
manifest nystagmus may be the only ones with a true statistics is the well-known fact that children with
congenital, occipital cortex--based, motor-sensory congenital esotropia are more likely to have a parent
fusion defect. or first-order relative with esotropia compared to chil-
When incomitant strabismus is present in a dren without congenital esotropia. Following this
patient with normal motor fusion potential, it can be study, we found no difference in the response to treat-
dealt with by assuming a head posture which results ment in those children with or without a parent with
in alignment of the eye. In these cases, it is the head subnormal binocular vision. This suggests to us that
that moves while the eyes remain fixed with fusion on defective stereo acuity is a subtle inborn defect which
the object of regard. Examples of this strategy could be a form fruste of congenital esotropia, but the
employed in Brown, superior oblique palsy, and true relationship between a minimal defect in stereo-
Duane are shown in Figure 10. acuity and normal alignment remains unexplained.
I believe that the presence of the keystone for On the other hand, asymmetric OKN is a reaction to
motor and ultimately sensory fusion is genetically ocular misalignment even as mild as minimal DVD
determined. Likewise, the absence of this keystone is and is therefore classified as a result, not a cause, of
genetic. The presence or absence of this characteris- congenital esotropia. The stereo acuity defect is
tic cannot be uncovered by clinical means at our dis- inborn and cortically based. The OKN defect is sec-
posal until approximately two to four months of age ondary and brain stem based.
when the system is programmed to complete the Another way to look at the picture of congeni-
scheme for the development of binocular alignment. tal esotropia in a graphic way and still retain the ele-
ments of sensory (afferent), motor (efferent), and sen-
Possible hereditary factors sory-motor fusion (link or keystone) components of
In an effort to learn more about possible hered- infantile esotropia is to picture a closed loop system
itary factors in congenital esotropia, we studied oth- (Figure 11).
erwise normal parents of children with congenital

137
Chapter 5

B B1

C C1

D D1

Figure 10
A By changing the foundation, the arch can maintain its C Duane syndrome, right eye
integrity provided the keystone of motor fusion is C1 This girl with Class II Duane turns her head toward the
intact. This is done when patients assume an appropri- normal side to achieve fusion.
ate head posture in: D Right superior oblique palsy
B Mild Brown syndrome, left eye D1 A chin down, head tilt left is the head posture assumed
B1 Eyes are aligned when head moves to position where to gain fusion by this child with congenital right superior
strabismus is maximum in Brown. oblique palsy.

138
Diagnostic categories & classification of strabismus

B C

*This infant may be indistinguishable from the normal by usual


clinical means.

Figure 11
A The nine components of the complete closed loop of C Congenital esotropia with a pathologic open loop.
binocular vision. D Mature normal loop.
B The immature open loop demonstrating unstable align-
ment.

139
Chapter 5

Characteristic No. (%)


Amblyopia 144 (35)
Anomalous head posture 26 (6)
Dissociated vertical deviation (DVD) 208 (51)
Overaction of inferior obliques (OAIO) 277 (68)
DVD and OAIO (combined) 171 (42)
Manifest nystagmus 62 (15)
Manifest-latent nystagmus 41 (10)
*Mean deviation at distance, 44 prism diopters (range, 5 to 100 prism diopters). Mean deviation at near, 49 prism diopters (range, 10 to
95 prism diopters)

Table 11 Characteristics of essential infantile esotropia* according to von Noorden (N=408)

The occurrence of associated finding with con- Ciancia syndrome is esotropia with limited
genital esotropia has been described according their abduction and manifest latent nystagmus of the
salient features by von Noorden (Table 11). He also abducting eye. These patients fixate with the adduct-
offers a differential diagnosis (Table 12). Other ed eye and turn their face to the side of the fixing eye.
descriptions of infantile esotropia have been offered This is called cross fixation. The primary congenital
by Lang, Adelstein, Cppers, and Ciancia. Harcourt defect is in the cortical motor fusion center, seen as
simply described congenital esotropia with and with- esotropia with manifest latent nystagmus secondary
out nystagmus. I prefer to recognize Ciancia syn- to brain stem response.
drome as congenital esotropia that has manifest latent Nystagmus blockage syndrome is manifest
nystagmus as the most prominent feature. Nystagmus congenital nystagmus damped by convergence. Both
blockage syndrome is manifest congenital nystagmus eyes are crossed. One eye is used for fixation and the
with a null in convergence. While several important face is turned toward the fixating eye. The primary
characteristics are associated with different types of defect is presumed to be in the brain stem.
esotropia in infancy, important clinical features of Latent and manifest latent nystagmus, dissoci-
congenital esotropia are strabismus (always),OKN ated vertical deviation, and amblyopia can occur as
asymmetry (always), nystagmus, and abnormal head later findings in any esotropia of infancy. In my
posture (sometimes). These features can be seen with experience, congenital esotropia without nystagmus
some or all of the findings listed as characteristics of is more likely to be aligned with one procedure con-
congenital esotropia in Table 11. sisting of an appropriate size bimedial rectus reces-
Early refractive esotropia, not included in this sion. Sprunger, et. al. have shown that in the presence
scheme, is usually easily distinguishable because of a
later onset, better fusion potential, and response to
correction of hyperopia. Other characteristics of con-
genital esotropia including amblyopia, DVD, and
oblique muscle dysfunction are secondary. The prin- Essential infantile esotropia*
cipal characteristics of esotropia in infancy can be Sixth nerve palsy
combined in a Venn diagram, which makes the rela- Nystagmus dampened by convergence
tionships of the early onset esodeviations easier to Esotropia with central nervous system anomalies
understand (Figure 12) (Table 13). (Downs syndrome, albinism, cerebral
Primary congenital esotropia has a moderate palsy, mental retardation, and the like)
to large angle esotropia. The patient may alternate or Refractive accommodative esotropia
prefer fixation with one eye (amblyopia). Sensory esotropia
Nystagmus, manifest or latent, and anomalous head Duane syndrome, type 1
posture are usually not present at the outset but may * congenital esotropia
occur later. The primary defect is presumed to be a
Mbius syndrome (EMH)
congenital defect in the central, cortical motor fusion
center.
Table 12 Differential diagnosis of infantile esotropia
according to von Noorden

140
Diagnostic categories & classification of strabismus

Figure 12 Venn diagram

Congenital esotropia syndrome (Lang)

1. Esotropia of early onset 4. Excyclodeviation of the nonfixing eye


2. Alternating sursumduction (DVD) 5. Abnormal head posture
3. Nystagmus 6. Slight hereditary influence

Essential infantile esotropia (von Noorden)

1. Onset--birth to 6 months Excessive adduction


2. Large angle, >30 prism diopters Dysfunction of oblique muscles
3. Stable angle Dissociated vertical deviation
4. Normal central nervous system 6. Initial alternation with cross fixation; potential
5. May be associated with: for normal binocular vision limited
Defective abduction

Nystagmus blockage (Adelstein-Cuppers)

1. Nystagmus on abduction 3. Straightening eyes under general anesthesia


2. Head turn in direction of fixing eye

Nystagmus blockage (von Noorden)

Manifest jerky nystagmus that decreases or disappears with esotropia, accommodative convergence
when sustaining fixation on an object distance or near.

Ciancia syndrome

1. Esotropia early onset in abduction and decreasing in adduction


2. Rather large angle deviation 5. Torticollis: the face is rotated toward the side
3. Bilateral limitation of abduction of the fixing eye.
4. Jerk nystagmus (manifest latent) with the quick 6. Hyperopia generally moderate or absent
phase toward the side of the fixing eye increasing

Table 13 Clinical Characteristics

141
Chapter 5

of nystagmus, it is more difficult to achieve alignment the fact that moderate amounts of uncorrected hyper-
in congenital esotropia. Both Ciancia syndrome and opia are usually accompanied by accommodation to
nystagmus blockage syndrome are more likely to achieve a clear image, and this would automatically
require additional surgery to achieve horizontal align- invoke accommodative convergence with the poten-
ment. Duane syndrome I is a truly congenital tial for elimination of or reduction of the exodevia-
esotropia but has a normal sensorimotor status and tion. Others have found a normal distribution of
with the proper head posture, in most cases, fusion is refractive errors in patients with intermittent
obtained. Surgery in cases of Duane syndrome is exotropia. For whatever reason, those patients I have
ordinarily done to improve head posture and primary treated for intermittent exotropia tend to have very lit-
position alignment. Even with successful surgery, tle, if any, hyperopia.
some strabismus remains.
Age of onset
Intermittent exotropia Clinically significant intermittent exotropia can
be seen in infants 6 months of age or even younger. A
Intermittent exotropia is a common, though more common age of onset is during the toddler peri-
enigmatic, form of strabismus that presents only a od, between 1 1/2 and 3 years of age. Parents may
modest diagnostic challenge but which requires con- observe that their child does not look at me. It is
siderable therapeutic acumen and tenacity. Patients usually difficult for parents to accurately describe this
with this type of strabismus, more than any other, type of strabismus. They often say that the eyes of the
demonstrate a duality of behavior in that they seem to child simply dont look right. At other times, par-
be completely normal during orthotropic interludes ents have said that they have seen their childs eyes
and totally turned off during periods of manifest turn in. After careful questioning it usually becomes
exodeviation. At the outset it should be stated clear that the parents are describing the recovery
exophoria; that is, a latent and usually small-angle movement from the exodeviation to the straight posi-
exodeviation, is not amenable to surgical treatment. tion--therefore, the turning in. The pattern of exode-
This latent deviation, which by definition is kept in viation over time is usually characterized by a steady
check under binocular conditions, can cause symp- progression with increasing periods of tropia during
toms of asthenopia best treated with orthoptic exer- the preschool years. However, in some cases the
cises, but not prism wear which will be eaten up. deviation remains stable and a few even improve.
These exercises can enhance motor fusion amplitudes Paradoxically, it has been shown by Archer that giv-
to control the deviation in both phoric and some ing full correction in some hyperopic patients can
intermittently tropic patients. In such cases, the lead to control of intermittent exotropia.
greater the motor fusion response for convergence,
the more effectively the deviation will be held in Pattern of deviation in the young
check with reduction or elimination of symptoms. Two characteristics of the intermittent
Orthoptic exercises can consist of near point of con- exotropia deviation in the preschool years are
vergence push up maneuvers, used for convergence (1) increasing likelihood of tropia when the child is
insufficiency that has a greater exodeviation at near, fatigued, day-dreaming, or inattentive and (2) closing
convergence enhancement with fusible targets, work- one eye in bright sunlight. The latter is an especially
ing against base-out prism, and use of over-minus interesting phenomenon. von Noorden and Wiggins
lenses that invoke accommodative convergence. have shown that fusional amplitudes decrease in the
In contrast to the exophoric patient, those majority of subjects, both strabismic and normal,
patients with intermittent exotropia who are tropic when tested with increasing illumination. It is also a
demonstrating a manifest deviation part of the time commonly observed phenomenon that most normal
and who require surgical correction occur in several people when in bright sunlight outdoors will close the
forms. They are classified on the basis of when the eye nearer to the sun, while unconsciously using the
eyes are deviated and in what field or distance of gaze nose in part to shade the fixating eye. If the sunny-
the deviation is greater. However, a strong undercur- side eye were exotropic, it would be all the more
rent of similarity connects all forms of intermittent exposed to the sun and therefore demanding closure
and latent exodeviations. for comfort. It is important to elicit this history of
closing one eye in bright sunlight when confirming a
Refractive error in intermittent diagnosis of intermittent exotropia in a young child.
exotropia When this question is asked of a parent and they
Patients with intermittent exotropia in our expe- respond in the affirmative, it lends a measure of cre-
rience have a low refractive error, either plus or dence to you, the examiner, and tends to confirm that
minus, with or without moderate astigmatism. This the exodeviation is present during these periods.
may be a valid observation, but it may also be due to

142
Diagnostic categories & classification of strabismus

Exodeviation in the newborn Classification of intermittent exotropia


When considering the onset and frequent pro- Intermittent exotropia has been classically
gression of intermittent exotropia in the young child, divided into four categories.
it is important to recognize that intermittent exotropia I. Basic type--The exodeviation after dissocia-
(with fixation of one eye) or divergence (without fix- tion is the same at distance and at near.
ation) is seen almost routinely in the normal newborn II. Divergence excess type--The distance
and infant. Archer leading our nursery eye examina- exodeviation is greater than the near deviation
tion team found that two thirds of newborns demon- by 15 prism diopters. The near point of con-
strated an exodeviation at some time during a motili- vergence in these patients is usually recessed.
ty examination. This exodeviation was seen in fewer III. Convergence insufficiency type--The near
children as examinations were done on older infants deviation intermittent exotropia is greater than
at monthly intervals. It was concluded from this the far deviation by 15 prism diopters. The near
study that exodeviations should not be considered point of convergence in these patients is usual-
clinically significant until after 6 to 8 months. ly recessed.
Esodeviations, in contrast to exodeviations, occurred IV. Simulated divergence excess type--The
in fewer than 3% of newborns and this deviation patient presents initially with a larger deviation
resolved earlier. Any esodeviation persisting beyond at distance compared to near. However, the
2 months is considered clinically significant. This near deviation is reduced on the basis of the
pattern of development of normal alignment makes it tonic near reflex and proximal convergence. In
difficult to make a firm diagnosis of intermittent order to differentiate between true and simulat-
exotropia before 6 to 8 months of age. ed divergence excess intermittent esotropia, the
patient should have one eye patched for 15 to
Pathophysiology of intermittent 30 minutes, after which careful alternate prism
exotropia and cover testing is carried out at near using an
accommodative target without allowing the
Why do the eyes go out? What is occurring in
patient to use both eyes together. If the near
the sensory system while the eyes are straight? When
deviation remains smaller, the individual has
they are out? Some things we know can be listed:
true divergence excess. On the other hand, if
First, when the eye is exodeviated in the manifest
the deviation increases to equal or nearly equal
phase of intermittent exotropia, the patient is symp-
the distance deviation, a diagnosis of simulated
tom free. There is no diplopia. Second, the binocu-
divergence excess--basic exotropia can be
lar peripheral field is enlarged when the eyes are devi-
made.
ated. However, this enlarged peripheral binocular
In addition to these categories of intermittent
field is more readily appreciated and potentially use-
exotropia, Knapp and Moore have emphasized the
ful in adults with constant exotropia. Third, a large
importance of lateral incomitance. These patients
central scotoma is present in the deviating eye during
will demonstrate a smaller exodeviation when alter-
the tropic phase. The suppression pattern and retinal
nate prism and cover testing is carried out in lateral
correspondence have been studied by Pratt-Johnson
gaze to either side in spite of having full abduction.
and Tillson, who found consistent harmonious anom-
Recognition of this is important, according to Knapp
alous correspondence. The role of so-called hemireti-
and Moore, because patients with 20% or more reduc-
nal suppression of the temporal retina has been
tion in exodeviation in lateral gaze are more likely to
stressed by Jampolsky. Older theories discuss
be overcorrected surgically if the amount of surgery is
anatomic orbital factors and the role of active diver-
done purely on the basis of measurements made in the
gence, the balance of forces at rest in the exodeviated
primary position. This finding of lateral gaze incomi-
position, and the role of the accommodative conver-
tance may be a measure of a patients convergence
gence/ accommodation ratio in overcoming an innate
response to the unnatural exercise of fixation in
tendency toward exodeviation. None of these expla-
extremes of lateral gaze maintained during prism and
nations is entirely satisfactory, though each may have
cover testing.
some validity.

Racial predilection A and V pattern with intermittent


exotropia
Intermittent exotropia is slightly more common
in the black race. This condition is also seen fre- Patients with intermittent exotropia also may
quently in fair-haired, blue-eyed Scandinavians and have an A or V pattern and in some instances may
in societies and cultures who are less involved in pro- have an associated hyperdeviation present in primary
longed near work. position when the eyes are dissociated. In other
cases, an X pattern may be present characterized by
an increase in the exodeviation in both up- and

143
Chapter 5

downgaze. The rules of treatment are consistent. If exodeviated or even straight during cover testing, at
oblique dysfunction is a cause of the A or V pattern, the expense of vision, which is fogged, though unde-
the obliques may be weakened. However, the superi- tected, by accommodative convergence.
or oblique should be weakened only if other surgical To obtain an accurate distance measurement
considerations such as downshift of the lateral or some prefer to measure while a patient fixates on a far
upshift of the medial rectus muscles would be con- distant object at infinity. To do this, the patient may
sidered ineffective, if no exodeviation is present in be asked to look out of a window at a distant chimney,
primary position and, of course, if definite overaction clock, tree, etc., while prism and cover testing is car-
of the superior oblique is demonstrated. Bilateral ried out to detect the maximum distance exodevia-
superior oblique weakening should be avoided in tion.
most A pattern patients who demonstrate fusion. In A short-term use of a patch can differentiate
most cases of intermittent exotropia with A or V, I simulated from true divergence excess. Use of alter-
prefer when possible to perform appropriate vertical nate patching for days or weeks in patients with inter-
shift of the horizontal recti, sometimes without reces- mittent exotropia has been said to result in a reduction
sion if no deviation is present in the primary position. in the exodeviation, but I have not employed this
X patterns will usually resolve with correction of the technique often.
primary position deviation after surgery done only on
the horizontal recti. I have not found it necessary to Parental observation
weaken all of the obliques, as has been suggested. During the initial examination of a child with
A, V, or X pattern deviations are more likely to intermittent exotropia, an important part of the
be seen in constant than in intermittent tropias. process is to actually demonstrate the deviation to the
parents. They should be shown the misalignment of
Combined horizontal and vertical the eyes just after dissociation and while the eyes are
deviation with intermittent exotropia exotropic. This is described to them as the devia-
If when the exodeviation is neutralized with tion. Next, the family observes the recovery move-
base-in prism during the prism and cover test the ver- ment. It has been important in our practice to send
tical deviation is eliminated, the vertical deviation families home from an initial examination of a child
(which may be called a dissociation vertical) may with intermittent exotropia with instructions to keep a
be ignored at the time of surgical correction. On the report card of their childs ocular alignment. At
other hand, if the vertical deviation persists after the subsequent examinations parents should report
horizontal deviation has been neutralized with prism, approximately how often the childs eyes are deviat-
it deserves to be treated with appropriate vertical rec- ed, under what conditions the childs eyes are deviat-
tus or inferior oblique surgery. Persisting vertical ed, how readily the childs eyes can be straightened or
deviation is more likely to occur with constant exode- recovery takes place, etc. Without this type of
viation. When combined vertical and horizontal sur- demonstration parents may bring such a child for
gery is done, this surgery usually consists of superior repeated examinations without ever really under-
rectus recession on the appropriate side. standing the true nature of the deviation!

Work-up of the patient with intermit- Nonsurgical treatment of intermittent


tent exotropia exotropia
Evaluation of the patient with intermittent Intermittent exotropia is amenable to orthoptic
exotropia should be carried out according to the exercises in certain cases. Convergence insufficiency
guidelines recommended (see chapter 4). Several in a teenager or an adult can be helped with consci-
characteristics unique to the intermittent exotropia entious performance of near point of convergence
patient and important in evaluation should be exercises, or in some cases, base-out prism may be
stressed. An accommodative target should be used used to stimulate convergence. On the other hand,
when doing the prism and cover test both in the dis- base-in prisms play only a small role in the treatment
tance and at near with the patient also wearing full of most cases of true intermittent exotropia because
correction. The reason for this is that patients can the patient usually has no symptoms, (except in cases
employ accommodative convergence in the distance of convergence insufficiency) only a deviation with
to maintain straight eyes at the cost a blurred image. suppression. Overcorrecting minus lenses may be
While reading a 20/20 target and wearing proper cor- used as an exercise to stimulate convergence to over-
rection, a patient will manifest the true exodeviation, come an intermittent exotropia. This type of treat-
free of the influence of accommodative convergence. ment would more likely be used in a case that has
Conversely, while fixing a distant fixation light been undercorrected at surgery in an effort to rescue a
(which is a nonaccommodative target) or a large tar- surgical attempt. Overcorrecting minus lenses used
get such as a 20/200 optotype, the eyes may be less in place of surgery are a form of procrastination.

144
Diagnostic categories & classification of strabismus

Symptomatic convergence insufficiency may be Most surgeons prefer to establish a course of progres-
treated with base-in prism. While of theoretical sion of the intermittent exotropia and then preform
value, I have not used weak or incomplete cyclople- surgery at the mid-preschool years, between 2 1/2 and
gia to stimulate accommodative convergence. 4 years of age or even later. However, exceptions to
this rule do occur; I have operated on a few patients
Surgical treatment of intermittent with intermittent exotropia who were under the age of
exotropia 1 year of age. In older children and adults with basic
intermittent exotropia, and adults with convergence
The surgical treatment of intermittent exotropia
insufficiency, surgery is done when the patient wish-
presents some philosophical problems. These
es, either because of problems with appearance or
patients have a lot going for them before anything is
from asthenopia, or both.
done. Vision is usually equal and normal in each eye,
sensory fusion with stereopsis and motor fusion Choice of muscles and amount of
amplitudes are present, and versions and ductions are
intact. In other words, the good intermittent
surgery
exotropia patient is very, very good. On the other Basic intermittent exotropia and pseudodiver-
hand, at times when the eyes are exodeviated, central gence basic intermittent exotropia are treated the
field binocular cooperation seems to be turned off. same way. The main choices surgically are between
One eye drifts out, suppression of the central field is bilateral lateral rectus recession and lateral rectus
profound, and that part of the visual experience is recession combined with medial rectus resection.
essentially ignored. The motility and sensory condi- While individual surgeons may have strong prefer-
tion in intermittent exotropia is certainly dynamic, ences for one technique. No strong evidence exists to
varying between having one turned off exodeviated support one technique over the other.
eye and having perfectly normal binocular coopera- For most cases, I prefer bilateral lateral rectus
tion. At the same time, a static surgical procedure is recession over recession-resection. The reason for
used for treatment. By static I mean that muscles are this is that the recession procedure is slightly more
recessed or shortened (resected) to alter an alignment physiologic than the resection procedure. In addition,
that is orthotropic much of the time. There is no evi- medial rectus resection can produce a lump under the
dence that this surgery has any specific effect on ver- conjunctiva. For larger deviations, it is necessary to
gences or fusional ability. With this apparent illogi- add to bilateral lateral rectus recession a medial rec-
cal application, the fit is not always perfect. Some tus resection or even bimedial rectus resection.
flexibility in treatment plan and diligence in the fol- A useful table for surgical numbers follows:*
low-up, especially with regard to nonsurgical inter- Bilateral lateral rectus recession
vention as well as surgical treatment, is necessary. 5.0 mm OU/20-25 prism diopters
6.0 mm OU/25-30 prism diopters
Timing of surgery 7.0 mm OU/30-40 prism diopters
Strabismologists disagree on the best time for 8.0 mm OU/40-50 prism diopters
surgery. Some prefer early surgery for intermittent Recession lateral rectus--resection medial
exotropia, saying that it is important to avoid pro- rectus
longed periods of suppression that can lead to deteri- 5.0 mm--*5.0 mm/20-25 prism diopters
oration of the normal fusion substrate. On the con- 6.0 mm--*6.0 mm/25-30 prism diopters
trary, others believe it is safe to follow these children 7.0 mm--*8.0 mm/30-40 prism diopters
with observation, stressing that patients are as good as 8.0 mm--*10.0 mm/40-50 prism diopters
they are at their best; the glass is half full, not half Three-muscle surgery
empty. Two arguments for delay are (1) overcorrec- 8.0 mm--*6.0 mm--8.0 mm/50-60 prism
tion is easier to deal with in an older, more coopera- diopters
tive patient and (2) some intermittent exotropia 8.0 mm--*8.0 mm--8.0 mm/60-75 prism
patients remain the same and a few even improve. diopters
The great fear in surgical treatment of intermittent Four-muscle surgery
exotropia is overcorrecting a very young patient and 8.0 mm--*8.0 mm--*8.0mm/70-85prism
converting this patient into a small-angle esotropia diopters
with loss of stereopsis and possibly producing ambly- 8.0 mm--*10.0 mm--*10.0 mm--8.0
opia. The majority of strabismologists do not consid- mm/85-100 prism diopters
er intermittent exotropia in the young patient between In most cases of intermittent exotropia requir-
ages approximately 1 and 3 years to be an emergency. ing surgery, the deviation is 50 prism diopters or less.

* Any time you read surgical numbers in this book, be aware that these are only my best approximation - a place to start. Each sur-
geons numbers must be his or her own! The second asterisk refers to medial rectus resection.

145
Chapter 5

Therefore, two-muscle surgery--either bilateral later- sary, these may be replaced with permanent prisms.
al rectus recession or recession of one lateral rectus Prolonged prism treatment of overcorrected intermit-
and resection of the same medial rectus muscle of the tent exotropia can produce excellent results. One
same eye--is sufficient. patient who was initially undercorrected with bilater-
al lateral rectus recession had bimedial rectus resec-
Divergence excess intermittent tion as a second procedure that produced 14 prism
exotropia diopters of esotropia postoperatively. She wore
steadily decreasing base-out prisms for three years
This condition is best treated with bilateral lateral rec-
before reverting to orthophoria with normal stereop-
tus recession. A table for surgery follows:
sis. Her father, a university professor of philosophy
5.0 mm OU/20-25 prism diopters
anxiously followed this long drawn out process, mak-
6.0 mm OU/25-30 prism diopters
ing it unforgettable. It is rare that a patient requires
7.0 mm OU/30-40 prism diopters
reoperation for overcorrection in cases where surgery
8.0 mm OU/40-50 prism diopters
has been done properly and ductions are full.
This surgery is done without regard for the near
However, incomitant esotropia occurring after sur-
deviation. In most cases, correction of the distance
gery for intermittent exotropia, especially after reces-
deviation has no adverse effect on the near deviation.
sion and resection has been done, may require reces-
However, in two very intense, highly motivated,
sion of a tight medial rectus muscle or advancement
teenage female patients the distance deviation could
of a weak lateral rectus muscle.
be repaired only at the expense of the near deviation.
Undercorrection of intermittent exotropia calls for
One patient requested to have her eyes over-corrected
reoperation based on the alignment with knowledge
to an esotropia when she was in college so that she
of the muscles previously operated. In general, if the
could read. She wore base-out prism for driving.
laterals have been recessed less than maximum, they
After she finished school and began to work as a
may be re-recessed. If the laterals have been recessed
nurse she did the opposite--that is, wanted her eyes
maximally, the medials may be resected, etc.
placed straight in the distance while she wore base-in
prism for reading at near. Convergence insufficiency
Convergence insufficiency intermittent exotropia is
Results of surgery for intermittent an entirely different situation. I have had some suc-
exotropia cess in treating this with bimedial rectus resection,
The treatment results for intermittent exotropia are but in other cases results have been disappointing,
not as easy to assess as in other forms of strabismus with virtually no change in alignment after surgery
treatment. In general, success is defined as convert- even in cases where an early overcorrection occurred.
ing an intermittent tropia to a phoria. Simply reduc- The amount of bimedial rectus resection should be
ing the angle of a persisting intermittent tropia is not small and symmetrical, ranging from 4 to 6 mm.
much help since the same sensory pattern, suppres- Some prefer a small recess-resect procedure to treat
sion when tropic, persists. In addition, the size can convergence insufficiency. Although this could cause
increase postoperatively. the patient to assume a slight face turn at near to
A desirable, but also sometimes worrisome, early maintain comfortable single binocular vision while
postoperative result is to convert an intermittent reading, the reward is that the patient can find an area
exotropia into a constant small angle esotropia. of fusion because the deviation is incomitant.
These patients experience diplopia postoperatively,
which may be treated with patching for a short time Does intermittent exotropia progress
or base-out prism if a longer period of treatment is to constant esotropia?
needed. These overcorrections eventually revert to
stable surgically corrected status in most cases. Any Intermittent exotropia can proceed to a constant devi-
patient (or parent) must be alerted to the fact that ation in some cases. This may be a reason to perform
diplopia from postoperative esotropia can be a good surgery in a timely manner. Perhaps in no other kind
sign. These patients are also given full plus glasses if strabismus is the surgeon more obligated to make
they have any hyperopia. Patients may also be given informed decisions ahead of time and to follow the
phospholine iodide to reduce accommodative conver- patient diligently postoperatively instituting appropri-
gence. ate and timely re-treatment. It cannot be overstressed
This treatment of the postoperative esotropia is con- that these patients are completely normal during peri-
tinued for several weeks or months. If the esotropia ods of alignment. The surgeon is obligated to reme-
with diplopia persists, it is treated with fully correct- dy the condition occurring when the patient is abnor-
ing Fresnel base-out prism on their present glasses or mal but to leave undisturbed the alignment present
on plano loaner glasses which are provided. If neces- during periods of normalcy. Herein lies the challenge
of the surgical treatment for intermittent exotropia.

146
Diagnostic categories & classification of strabismus

The mechanical restriction to elevation in


A professional football player who I adduction causing Brown syndrome may occur on a
examined had a large angle intermittent, nearly congenital unknown basis, after trauma, from iatro-
constant, exotropia at a routine eye examina- genic causes, with a cyst of the tendon, and with
tion. He was told that his eyes could be inflammation (see page 232). In essence, Brown syn-
straightened. No, Doc, he replied, I like the drome can result from anything that prevents the nor-
way my eyes are because I can see people run- mal increase in the trochlear--superior oblique tendon
ning up behind me. At the next years eye insertional distance, even including mechanical
examination he said, Doc, could I catch a pass restriction elsewhere on the globe.
better if my eyes were straight? When the Brown syndrome may have a hereditary basis
answer was yes, he consented to have his eyes in some cases. It has been reported in a parent and
aligned surgically. child and in siblings. I examined a young woman
This story points out the plus of exodevi- with Brown syndrome who stated that her maternal
ation, the enlarged binocular visual field. It aunt and grandmother were similarly affected.
also points out the downside of exodeviation, Acquired Brown syndrome caused by inflam-
reduced binocular cooperation - fusion. Adults mation is usually associated with pain or tenderness
having surgical treatment of constant exotropia in the area of the trochlea. Brown syndrome may also
should be told that they will experience tunnel occur because of a painless cyst around the trochlea.
vision with straight eyes, but this is transient. Brown syndrome caused by a cyst or inflammation
may be intermittent, recurrent, or episodic. Palpation
in the area of the trochlea may reveal a cyst or ten-
derness typical of an inflammatory mass. In some
cases a click can be heard, usually by the patient, with
Brown syndrome the click occurring as the cyst or mass on the supe-
Brown syndrome, characterized by a mechani- rior oblique tendon passes into or out of the trochlea.
cal limitation of elevation in adduction, was original- Such a click has been reported in an eye that had been
ly described by H.W. Brown in 1950 as the superior enucleated! In this case, removal of the eye did not
oblique tendon sheath syndrome. Brown syndrome is change the tendon-trochlea relationship sufficiently
now more broadly considered as an inability to fully to stop the click. Brown syndrome from an inflam-
elevate the eye in adduction, and to a lesser extent in matory response can be treated with injection of sol-
the primary position and sometimes abduction. This uble steroid into the trochlear area (but not into the
is caused in most cases, but not exclusively by a trochlea). This can produce dramatic relief, but the
restriction related to the superior oblique tendon Brown syndrome can recur, requiring repeat treat-
and/or trochlea. Because of this, the chin may be ment. Fortunately, in most cases, inflammatory
habitually elevated to capitalize on normal binocular- Brown syndrome is self-limiting. On the other hand,
ity in gaze down and toward the affected side. Brown recurring Brown syndrome from a palpable cyst
syndrome is differentiated from inferior oblique pare- tends to persist. We have successfully treated such
sis (which is rare) on the basis of restricted passive cases by excision of a cyst of the reflected tendon.
ductions on attempted elevation in adduction occur- Rao has reported Brown syndrome occurring from a
ring in Brown syndrome. Brown syndrome can be parasite - worm - lodging in the trochlea. This worm
unilateral or bilateral and may be associated with has been confirmed by MRI. This condition responds
superior oblique overaction. Some patients with mild favorably to antihelmenthic treatment.
congenital Brown syndrome have normal head pos- I have not exposed the trochlea itself in any sur-
ture and are symptom free. gery for Brown syndrome. Surgery for Brown syn-
While originally thought to be due to a short drome is limited to the reflected tendon, up to but not
superior oblique tendon sheath, the collective experi- including the trochlea.
ence of many surgeons gained during surgical treat- Most Brown syndrome is idiopathic and con-
ment of Brown syndrome has cast doubt on this etiol- genital. Surgical treatment is indicated for the fol-
ogy even to the point of denying that the superior lowing: (1) chin elevation, (2) hypotropia in primary
oblique tendon even has a sheath. Instead of having position, (3) diplopia, or (4) marked shootdown with
a true sheath, the tendon passes obliquely through adduction. As children with Brown syndrome grow
Tenons fascia between the trochlea and the union of taller, more of their world is straight ahead and below,
the superior oblique tendon with the capsule of the meaning the implication of Brown syndrome is less.
superior rectus before inserting into the sclera. This Patients with untreated mild congenital Brown syn-
passage through multiple orbital fascial layers pres- drome are seldom bothered by this condition as
ents many opportunities for anomalous tissue rela- adults. The same degree of acquired, especially iatro-
tionships, but actual orbital facial abnormality as a genic, Brown syndrome usually causes bothersome
cause of Brown syndrome is rare in my experience. symptoms in adults. Patients with congenital Brown

147
Chapter 5

syndrome are in marked contrast to patients with increased normally in upgaze during elevation in
Duane syndrome who tend to become increasingly adduction. During a period of 10 years, I treated 59
troubled by their strabismus in adulthood, far out of patients with tuck or resection of the superior
proportion to what seems a minimal deviation while oblique. Nine of these 59 or 17% had a postoperative
patients with congenital Brown syndrome are not par- Brown syndrome so severe that a second surgery was
ticularly bothered as adults. needed to take down the tuck. Since that series of
When treating congenital Brown syndrome sur- cases, I have learned about the wide variation in the
gically we do a cuffed limbal incision, hook the superior oblique tendon, especially in congenital
superior rectus, and inspect the superior oblique ten- superior oblique palsy. With this knowledge, superi-
don carefully, beginning at the insertion and continu- or oblique tuck and resection are done on a more
ing to the trochlear cuff. In most cases, in order to selective basis, being reserved for congenital superior
have better exposure, it helps to disinsert the superior oblique palsy with a loose or anomalously inserted
rectus and replace it later. Forced ductions are repeat- tendon confirmed by the superior oblique traction test
ed often during the process of identifying the tendon and reconfirmed at surgery.
to determine the cause of limited elevation. The best Traumatic Brown syndrome is difficult to treat.
treatment for congenital Brown varies according to The scarring associated with trauma in the area of the
the surgeons experience and may be one of the least superior oblique tendon and trochlea is difficult if not
agreed upon aspects of strabismus surgery. This is impossible to totally eradicate to produce free move-
not too surprising because freeing a mechanical ment because of the mechanical restriction which
restriction while simultaneously maintaining ade- usually affects both up- and downgaze. Traumatic
quate rotations in all directions may be the strabismus Brown syndrome coexisting with superior oblique
surgeons greatest challenge. Most surgeons believe underaction has been called canine tooth syndrome
that the least amount of surgery that will free ductions by Knapp. It is due to (1) local trauma restricting
is the best. For example, one surgical option is disin- both upgaze and downgaze, (2) local trauma restrict-
sertion of the posterior seven-eights of the superior ing upgaze and fourth nerve palsy restricting
oblique insertion at least as a first try. If this frees downgaze, or (3) iatrogenic Brown syndrome restrict-
passive ductions at surgery, no more surgery is done. ing upgaze and residual fourth nerve palsy restricting
This works very well in a few cases, probably downgaze (Figure 13).
because the posterior-medial fibers of the tendon have Before undertaking surgery for Brown syn-
mainly a vertical effect, but the procedure can fail drome, the surgeon must first confirm the diagnosis
with return of the Brown in spite of demonstrating by demonstrating restricted forced ductions at eleva-
free forced ductions at the conclusion of surgery. tion in adduction then:
Other surgical options, all involving the superior 1. Make sure there is a good reason for under-
oblique complex, include freeing the fascia surround- taking surgery, such as diplopia, hypertropia,
ing the tendon, recession of the tendon, tenotomy or a bothersome head posture
near the insertion, tenotomy between the superior rec- 2. Prepare the patient to have lowered expecta-
tus and trochlea, and use of a silicone expander. All tions
techniques have their advocates. The fact that there 3. Be ready for a possible second surgery,
are so many choices leads to the undeniable conclu- including treatment for iatrogenic superior
sion that no specific alternative is the best for all oblique palsy, especially if a superior oblique
cases. tenectomy is done.
Regardless of how the superior oblique is 4. Do not weaken the inferior oblique at the first
weakened, the risk of postoperative superior oblique procedure.
palsy exists. Crawford found this frequently. Parks 5. Remember that the little people grow up,
suggests simultaneous inferior oblique weakening to meaning that restricted upgaze could become
treat the presumed superior oblique palsy caused by less of a factor.
superior oblique weakening to treat the Brown syn- 6. Apply any other good points that experience
drome. However, Sprunger et al., found only a third has taught.
of patients having superior oblique tenectomy for
Brown syndrome had superior oblique palsy requir-
ing inferior oblique weakening. They suggest doing
inferior oblique weakening at a second procedure and
only if needed.
Iatrogenic Brown syndrome results from exces-
sive shortening the superior oblique tendon when
treating superior oblique palsy with a resection or a
tuck. It occurs because the distance between the
trochlea and the superior oblique insertion cannot be

148
Diagnostic categories & classification of strabismus

or inferred

Figure 13 Scheme of Brown Syndrome

Duane syndrome I. Marked limitation of abduction (or


absence), normal or slightly defective
Duane syndrome, or Stilling-Turk-Duane syn- adduction, narrowing of the palpebral fis-
drome, was described before the turn of the 20th cen- sure and retraction of the globe on adduc-
tury. It is characterized by limitation of abduction or tion, widening of the fissure on attempted
adduction, narrowing of the fissure with enophthal- abduction with esotropia in the primary
mos, and face turn all of which vary according to the position, and head turn toward the
class of Duane. The etiology of Duane syndrome is involved side.
agenesis of the sixth nerve nucleus in the brain stem II. Limitation or absence of adduction,
on the involved side plus misdirection of the third exotropia of the affected eye, normal or
nerve innervation to the medial rectus. This misdi- reduced abduction, narrowing of the fis-
rected medial rectus innervation goes to the lateral sure on attempted adduction, often with
rectus in the orbit. The sixth nerve nucleus patholo- upshoot or downshoot, and face turn
gy was found originally in an autopsy specimen toward the normal eye.
obtained at his request from an ophthalmologist, Otto III. Limited abduction and adduction, retrac-
Pranjen, of the Mayo Clinic. Pranjen, who had Duane tion of the globe, and narrowing of the fis-
syndrome, willed has brain for study. Later, Miller sure on attempted adduction with straight
and Green confirmed the third nerve misdirection. or nearly straight eyes in the primary posi-
They found third nerve fibers intended for the medial tion and often with upshoot or downshoot.
rectus going to the lateral rectus in the orbit during There is usually no face turn.
post-mortem studies of a patient examined before It may be easier to keep the different types of Duane
death and confirmed to have Duane. Earlier, Huber syndrome in mind by simply describing the primary
had demonstrated co-firing of the medial and lateral position alignment. The majority of patients with
recti with electromyography. Duane syndrome have an esotropia of the involved
Duane syndrome has been classified by Huber eye when the head is straightened. When the patient
according to alignment and ocular rotations into is allowed to assume the most comfortable head pos-
classes I, II and III. ture, the face invariable turns toward the involved

149
Chapter 5

side and the eyes toward the opposite side. In most able head posture, severe up- and downshoot of the
cases, these patients are able to recognize normal or affected eye in adduction, and severe enophthalmos.
near-normal stereopsis and rarely have amblyopia. The most common type of Duane syndrome--the type
Enophthalmos with fissure narrowing usually occurs with moderate esotropia in the primary position along
in the involved eye only during attempts to extreme with face turn toward the involved eye--is best treat-
adduction. Abduction in the involved eye is typically ed with a small recession of the medial rectus of the
just beyond the midline. Upshoot and downshoot are involved eye. Other types of Duane syndrome are
not a prominent feature. treated with the aim of aligning the eyes in the pri-
Other patients with Duane syndrome are either mary position. It is a good rule to avoid resection of
orthotropic in the primary position or have an the lateral rectus muscle in Duane syndrome. This
exotropia in the primary position. The primary posi- can lead to worsening of enophthalmos and up- and
tion alignment seems to depend on the tightness or downshoot. Some have advocated modified full ten-
tone of the lateral rectus muscle. The tighter the later- don transfer of the superior and inferior rectus to the
al rectus muscle the less esotropic the patient (the lateral rectus to increase the field of binocular vision.
straighter or more exotropic the involved eye), and I have not done this procedure, but it has been done
the greater the enophthalmos on attempts at adduction safely and there could be indications for doing this.
in the involved eye. These patients with a tight later- When up- and downshoot are the main problems, it
al rectus are also more likely to have up and/or down- may be appropriate to recess both the medial and lat-
shoot of the involved eye in adduction. This holds eral recti of the involved eye and also the medial or
true except in patients who have a very large-angle lateral rectus of the fellow eye. Other techniques for
exotropia with Duane syndrome. These patients can treating the up- and downshoot include posterior fix-
demonstrate simultaneous abduction when looking ation suture of the lateral rectus muscle to keep it
toward the sound eye. This is presumably due to the from slipping upward and downward and also Y split
mechanical advantage assumed by the lateral rectus of the insertion of the lateral rectus muscle (Table 14).
during co-contraction with the eye already widely When treating Duane syndrome, it is essential
exodeviated. This could be called class IV. to know and also to inform the patient that this con-
The upshoot or downshoot in Duane is not due dition cannot be eliminated; however, the signs and
to over- or undercorrection of the oblique muscles but symptoms can be improved by appropriate surgery.
is instead caused by a taut wire effect with the eye
either slipping above or below the midline under the Practical classification of Duane
influence of the extreme tension produced by medial syndrome
rectus contraction against a co-contracting lateral rec-
tus. The extreme type of exotropic Duane syndrome Esotropic Duane - Huber I
with simultaneous abduction was originally called 1. Esotropia with head straight
perversion of the extraocular muscles. It is more 2. Face turn to involved side
accurately described as simultaneous abduction. This 3. Limited abduction
condition is rare, but I have seen a half dozen cases. 4. Near normal adduction
Regardless of the variations in clinical appear- 5. Mild enophthalmos and fissure narrowing
ance, all Duane syndrome patients appear to have a on adduction (but may be severe)
common etiology, just a varied expression. The con- 6. Sensory examination usually normal
dition seems to be slightly more prevalent in girls and
in the left eye. It is usually unilateral but may be Exotropic Duane - Huber II
bilateral. In bilateral Duane the rules for head posi- 1. Face turn toward normal side
tion do not follow. Duane syndrome has been 2. Limitation of adduction and no or min-
described as a genetic condition occurring in siblings imal limitation of abduction
and in consecutive generations. Duane syndrome is 3. Marked upshoot and downshoot on
also associated with craniofacial-mandibular cleft attempted adduction
anomalies including Goldenhar and Wildervanck syn- 4. Enophthalmos and fissure narrowing on
dromes. Children with Duane syndrome rarely com- attempted adduction, usually with up-
plain. They are usually brought in for examination shoot and downshoot
because of the head turn or strabismus, but often par- 5. More likely to suppress
ents are really unsure about the specific problem.
They simply suspect that there is something wrong. Straight Duane - Huber III
On the other hand, adults with Duane syndrome often 1. Limited abduction and adduction
complain bitterly of asthenopia, intermittent diplopia, 2. Marked narrowing of fissure on attempt
and a general feeling of being ill at ease. ed adduction with enophthalmos
Indications for surgery for Duane syndrome 3. Upshoot and downshoot on attempted
include strabismus in the primary position, unaccept- adduction

150
Diagnostic categories & classification of strabismus

Diagnosis Surgery

Esotropic Duane I Recess medial rectus on involved side* -- consider poste-


rior fixation suture contralateral medial rectus

Esotropic Duane with severe enophthalmos I Recess medial and lateral recti of involved eye, medial
rectus recession or posterior fixation suture on contralat-
eral eye if larger esotropia

Straight-eye Duane with up- and downshoot III Recess medial and lateral recti of involved eye if enoph-
thalmos severe; consider Y split of lateral rectus or poste-
rior fixation suture of lateral rectus (both with or without
recession)
Exotropic Duane II Recess lateral rectus of involved eye; can add Y split;
may require larger lateral rectus recession of uninvolved
eye.

Simultaneous abduction IV Recess both lateral recti; emphasis on the involved side;
may need to resect medial rectus of involved eye

* Extraocular muscle transfer shifting the vertical recti adjacent to the lateral rectus insertion of the involved eye has been done and may
be a viable option for mild esotropic Duane.
Table 14 Duane treatment guidelines

4. Straight or nearly straight head posture


5. Normal or near normal sensory examina- Most common findings of Duane syndrome
tion in 50 consecutive cases seen in consultation
with ORBIS Telemedicine
Simultaneous abduction - EMH IV
1. Large-angle exotropia
2. Face turn to uninvolved side Female 32/50
3. No adduction Left eye 25/45*
4. Simultaneous abduction looking toward V pattern 21/50
uninvolved side Upshoot 24/50
5. Usually suppresses Minimal amblyopia 6/50
I Head turn toward involved side
II Head turn toward normal
Superior oblique palsy III Head posture straight
Superior oblique palsy is the most commonly IV Head turn toward normal
occurring isolated cranial nerve palsy seen by the Class I common 25/50
strabismologist. However, I do not know of any reli- Class II nearly as common 16/50
able figures comparing the relative occurrence of Class III less common 8/50
fourth and sixth nerve palsies. Certainly, third nerve Class IV rare 1/50
palsy is rarer than either fourth or sixth nerve palsy. Hyperopia 23/50
In some practices, particularly those of the neuro- Right eye 20/50
ophthalmologist, sixth nerve palsy may be the most Bilateral 5/50
common.
*excluding 5 bilateral cases
Superior oblique palsy can occur from trauma,
congenital causes, a microvascular accident, and from
a mass lesion. These etiologies are differentiated pri-
marily on the basis of history with additional infor-
mation obtained from physical and imaging findings.

151
Chapter 5

The fourth nerve nucleus is in the rostral part of Brown syndrome after superior oblique strengthen-
the brain stem in the tectum. The nerve fibers emerge ing.
from the nucleus dorsally and decussate. The fibers Double Maddox rod torsion. Seeing a tilted
then pass through the tentorium as delicate fibrils. line on testing with double Maddox rod is very sup-
They course into the orbit through the superior orbital portive of the diagnosis of superior oblique palsy.
fissure where they have as their sole purpose the This usually means that the superior oblique palsy is
innervation of the superior oblique muscle. These acquired.
delicate fibrils are vulnerable to violent to-and-fro Overaction of the contralateral superior
motion of the brain, such as occurs with a sudden oblique. Underaction of the ipsilateral inferior rectus
deceleration in an automobile accident or similar (so-called fixation duress because it is working
head trauma. against a contracted antagonist) and overaction of the
contralateral superior oblique, its yoke, occurs in
Patient presentation longstanding superior oblique palsy with contracture
History. The patient or parents of the patient of the ipsilateral superior rectus.
frequently either describe an acute event (such as a Double Maddox rod torsion greater than 15
motor vehicle accident) that is likely to be the cause degrees. This is a strong indication of bilateral supe-
of a traumatic fourth nerve palsy or report a history of rior oblique palsy.
diplopia, asthenopia, or anomalous head posture, Bielschowsky head tilt test. This test is consid-
often present for many years or for life. Occasionally, ered positive for superior oblique palsy when the ver-
old pictures demonstrating a head tilt and chin tical deviation increases with the head tilted toward
depression are useful in supporting the diagnosis of the higher eye. If the Bielschowsky head tilt test
congenital superior oblique palsy. reverses, then a bilateral superior oblique palsy is sus-
Head posture. The usual head posture in supe- pected. If the Bielschowsky head tilt test does not
rior oblique palsy is the head tilted to the opposite reverse but is reduced to no or very little hypertropia
side with the chin depressed. Actually, the head in the same direction on tilt to the side opposite the
moves where the eye cannot be moved by the paretic paretic superior oblique, a masked bilateral superior
superior oblique. This is the rule when fusion poten- oblique palsy may be suspected.
tial is present in a person with incomitant strabismus. Fundus torsion. Torsion may be noted during
With the head in this posture, the eyes look upward examination with the indirect ophthalmoscope. If the
and to the opposite side, completely out of (opposite) macula is rotated downward or clockwise in the left
the field of action of the paretic muscle. In a very eye and counterclockwise in the right eye, so that the
small percentage of patients, the head posture may be macula is below a line drawn parallel to the orbit
in the opposite direction, presumably to maximize the floor and temporal from the lower disc margin, tor-
separation of diplopia and make it easier to suppress. sion can be inferred. This is confirmed if the macula
Torticollis. Neck contracture can occur in very is also shown to be roughly equidistant between the
young children with superior oblique palsy. temporal arcades while in its lower position.
However, neck contracture from superior oblique Inhibitional palsy of the contralateral antago-
palsy does not occur before the child sits up and/or nist. When the eye with the paretic superior oblique
walks. The head tilt from superior oblique palsy does is used for fixation the yoke inferior rectus in the con-
not occur with the child supine or prone but only tralateral eye receives extra innervation. Its antago-
when the child is vertically oriented, sitting, standing nist, the superior rectus and also the levator palpebri
up or walking. on that side are inhibited resulting in hypotropia and
Motility. Versions are an extremely important more importantly, pseudoptosis. When the normal
part of the diagnosis of superior oblique palsy. The eye takes up fixation, the ptosis disappears.
most tell-tale finding is inferior oblique overaction, Other indicators.
and to a lesser extent superior oblique underaction, 1. Diagnostic position prism and cover testing is
which occurs to varying degrees. Sometimes the more important for quantification of the deviation
superior oblique underaction is slight or undetectable. than it is for diagnosis.
Diplopia. Vertical diplopia is a common com- 2. Facial asymmetry is seen commonly in cases of
plaint in adult patients but rare in children. congenital superior oblique palsy. The face is
Asthenopia is also common in adults. This may take always fuller on the side of the paretic muscle.
the form of neck ache while reading. The reason for this is the abnormal head posture
Chin depression. In the presence of a V pattern assumed to maintain single binocular vision.
the chin is often down. This occurs with bilateral 3. Horizontal strabismus can occur in addition to the
superior oblique palsy. superior oblique palsy.
Cyclodiplopia. Spontaneous complaint of 4. Amblyopia in the presence of congenital superior
cyclodiplopia is a common sign of acquired bilateral oblique palsy may indicate an abnormal or even
superior oblique palsy. It also occurs in iatrogenic absent superior oblique.

152
Diagnostic categories & classification of strabismus

When the preceding considerations have been Congenital superior oblique palsy
dealt with and when full measurements have been
completed, especially prism and cover testing in the There is usually no history of trauma.
diagnostic positions, double Maddox rod testing, and The condition is long-standing and character-
the head tilt test, it is possible to diagnose, classify, ized by a large head tilt and supported by fam-
and establish a treatment plan for a patient with supe- ily pictures showing a head tilt.
rior oblique palsy. In cases of absence of the superior oblique ten-
don, amblyopia and horizontal strabismus are
Acquired superior oblique palsy common.
Facial asymmetry is common in all types of
Patients with acquired unilateral palsy will usu- congenital superior oblique palsy. The face is
ally have the following characteristics: fuller on the involved side.
A discrete history of onset There is frequently no torsion measured with
Complaint of intermittent vertical diplopia the double Maddox rod.
Head tilt and chin depression with a comment, There are fewer complaints of diplopia in con-
I see better if I assume this head position genital compared to acquired superior oblique
Measurable torsion with the double Maddox palsy.
rod, less than 15 degrees In the operating room, patients with congenital
A vertical deviation usually less than 20 prism superior oblique palsy a frequently found to have a
diopters (deviation may be greater at near and lax superior oblique traction test (see page 97). At
in longstanding cases). exploration of a superior oblique tendon that was
Bilateral acquired superior oblique palsy differs found to be loose or lax with the traction test, an
in that a V pattern is the rule; single vision is more anomalous superior oblique tendon will be noted to
likely to occur in upgaze with chin down; be either too long, inserted in the wrong place, or
Bielschowsky test is bilaterally positive; that is, right absent.
hyper with right tilt and left hyper with left tilt or the
hyper may disappear or nearly so on head tilt to one Superior oblique treatment
side; and the Maddox rod frequently shows a classification
cyclotropia of greater than 15 degrees.
Unilateral superior oblique palsy from a Treatment is based on prism cover measure-
microvascular accident is usually much smaller ment findings, torsion, and the results of superior
amplitude than unilateral superior oblique palsy from oblique traction testing indicating the state of the ten-
trauma. These patients usually have a vertical devia- don. Hatched areas shown in the diagrams on the fol-
tion in the neighborhood of 5 to 10 prism diopters and lowing pages represent the field of greater deviation
are older, being more often in the seventh or eighth and assume left superior oblique palsy. The pattern of
decade, and they complain of diplopia. They may not deviation is the examiners view.
demonstrate a head tilt. These patients deserve a med- The scheme described here is that proposed by
ical/neurological work up for hypertension, diabetes, Philip Knapp in 1971. It remains, with a few modifi-
etc. cations, valid today (Figure 14).
In the operating room, patients with acquired
superior oblique palsy, either unilateral or bilateral,
will usually be found to have a normal superior
oblique tendon on the traction test. The tendon is
very easily felt, and the traction test is usually bilater-
ally symmetrical in unilateral disease.

153
Chapter 5

Figure 14 Superior oblique palsy -- scheme for etiology

Class I Class II
Knapp I--overaction of antagonist inferior Knapp II--underaction of the paretic superior
oblique with deviation about 20 prism diopters or less oblique with the deviation greater in the field of
in the field of action of the antagonist; this is a com- action of the paretic superior oblique seen mostly in
mon pattern for both acquired and congenital superi- smaller angle, acquired microvascular superior
or oblique palsy oblique palsy. This is best treated with prism and
Surgery. Weaken antagonist inferior oblique. time. A larger angle deviation with this pattern can
This is the safest surgical procedure for any superi- occur in congenital absence of the superior oblique
or oblique palsy. tendon. If a pattern like this emerges in a congenital
superior oblique palsy with facial asymmetry and
pronounced superior oblique underaction, superior
oblique traction testing followed by exploration of the
superior oblique will lead to the appropriate surgical
plan which could include superior oblique tuck, infe-
rior oblique weakening, or yoke inferior rectus weak-
ening depending on the angle and the state of the
superior oblique.

154
Diagnostic categories & classification of strabismus

Class III Class V


Knapp III--In this class, the deviation is A hyperdeviation across the bottom can be the
approximately equal in the field of the paretic superi- pattern in a long-standing acquired superior oblique
or oblique and the antagonist inferior oblique. palsy pattern.
Surgery. If the deviation is less than 20 prism Surgery. If the vertical deviation is around 20
diopters in the field of greater deviation, only the prism diopters, the ipsilateral superior rectus is
antagonist inferior oblique weakening is done. If it is recessed and either the superior oblique is tucked, the
greater than 20 prism diopters and the superior yoke inferior rectus is recessed or the ipsilateral infe-
oblique traction test reveals a lax tendon, and con- rior oblique is weakened. The recommendation to
genital superior oblique palsy is diagnosed, a tuck of weaken the contralateral superior oblique along with
the superior oblique can be performed; if the superior a tuck of the involved superior oblique originally rec-
oblique tendon is tight, the yoke muscle, the con- ommended by Knapp is a bad idea. Only a lax ten-
tralateral inferior rectus, is recessed. If the surgeon don should be tucked and be wary of weakening the
chooses not to tuck the superior oblique tendon, even normal superior oblique in a fusing patient!
though lax, recession of the yoke can be done.

Class VI
Bilateral superior oblique palsy
This condition is characterized by:
1. History of trauma
2. Spontaneous torsional diplopia
Class IV 3. Usually >15 degrees torsion with double
This common pattern of hyperdeviation which Maddox rod testing
is class III demonstrates a spread of hyperdeviation 4. V pattern
across the bottom occurring because of tightness of 5. Reversing Bielschowsky (or nearly revers-
the ipsilateral superior rectus. ing Bielschowsky) test
Surgery. If the deviation is 20 prism diopters Surgery. There is little agreement among
or less, weakening of the antagonist inferior oblique experts when it comes to surgical treatment of bilat-
and ipsilateral superior rectus is effective. If the devi- eral superior oblique palsy. Bilateral weakening of
ation is greater, the superior oblique tendon can be the yoke inferior obliques is favored by some to treat
tucked if it is loose or the yoke inferior rectus can be the V and the torsion. Others do a bilateral reces-
recessed if the superior oblique tendon is normal. sion of the inferior recti. The strength of either pro-
cedure is that the weakening is done on a normal mus-
cle, one is a yoke and the other an antagonist.
Bilateral weakening of the antagonist inferior
obliques likewise treats the V and the torsion, but
depends on getting more out of a paretic muscle.
Antero-lateral shift of the superior oblique (Harada-
Ito) treats the torsion. For the V, downshift of the
medial recti can be done. The superior oblique ten-
don should not be tucked.

155
Chapter 5

Congenital superior oblique palsy on


an anatomic basis
When a congenital superior oblique palsy with
an anomalous tendon is encountered first by finding a
loose superior oblique traction test and then after the
tendon is exposed, strengthening of the tendon is
carried out on the basis of what is found.
In 190 cases of superior oblique palsy treated by
our group, 87% of those diagnosed as congenital had
Class VII an anomalous superior oblique tendon. The majority
of these had a redundant tendon that is class I anatom-
Brown syndrome with superior ic superior oblique palsy. These are the superior
oblique palsy patients who may be treated with supe-
oblique underaction (canine tooth) rior oblique tuck or resection if the tendon is suffi-
This condition is characterized by: ciently loose or lax. A legitimate question is, When
1. History of trauma to the trochlea with lax tendons are found in cases of V pattern congen-
mechanical restriction in upgaze and ital esotropia is this a form of bilateral congenital
downgaze. superior oblique palsy? (Figure 15). Congenital
2. Trauma to the trochlea restricting upgaze and anatomic superior oblique palsy is always associat-
residual superior oblique palsy restricting ed with a lax superior oblique traction test.
downgaze. The patient with superior oblique palsy must be
3. Can occur as an iatrogenic Brown after supe- managed with a comprehensive program of diagnosis
rior oblique tuck with residual superior and treatment.
oblique underaction. In summary, superior oblique palsy treatment
This problem is difficult to treat. My treatment sug- consists of:
gestions are: 1. Fresnel prism for acute symptomatic
1. None -- if eyes are aligned around primary microvascular fourth nerve palsy
2. Yoke inferior rectus recession -- if ipsilateral 2. Permanent prism for selected small-angle
hyper acquired small angle fourth nerve palsy
3. Take down tuck, if caused by a too tight tuck 3. Surgery according to angle and pattern for
4. Free superior oblique restriction if ipsilateral unilateral acquired fourth nerve palsy; avoid
hypo superior oblique tendon tuck; an anterior
transfer may be done if torsion is the main
problem
4. In congenital superior oblique palsy with ten-
don anomaly surgery is concentrated on the
antagonist, the yoke, and the lax superior
oblique tendon.
5. The safest surgical procedure in any superior
oblique palsy is weakening of the antagonist
inferior oblique
6. In longstanding superior oblique palsy weak-
en a tight ipsilateral superior rectus if there is
underaction of the ipsilateral inferior rectus
and/or overaction of the contralateral superior
oblique.
7. Bilateral superior oblique palsy can be treated
with weakening of the yoke, weakening of the
antagonist, and antero-lateral shift of the
superior oblique.

156
Diagnostic categories & classification of strabismus

8. Be aware of the possibility of masked bilater- established congenital disease, or less clear presumed
al superior oblique palsy. If the Bielschowsky microvascular disease in an elderly individual. In the
head tilt reverses, or nearly does, be suspi- last instance, an evaluation by an internist for hyper-
cious. Either treat this as a unilateral superi- tension and/or diabetes is needed. Extensive imaging
or oblique palsy and expect to do a second with CT or MRI or lumbar puncture and EEG studies
procedure or do two muscles on the more rarely accomplish anything useful for the usual supe-
involved side and one muscle on the masked rior oblique palsy patient. In my opinion, extensive
side according to the scheme presented. testing of the patient with fourth nerve palsy should
Work-up of a patient with acquired superior be done only if indications other than the fourth nerve
oblique palsy should in most cases be kept to a mini- palsy itself are noted. By that I mean other significant
mum. The etiology is usually clear-cut trauma, well- neurologic signs or symptoms.

Class I. Redundant tendon Class II. Misdirected tendon

Class III. Tendon in Tenons Class IV. Absent tendon

Figure 15
Description of the superior oblique tendon in anatomic congenital superior oblique palsy.

157
Chapter 5

TABLE I: PATIENT DEMOGRAPHICS AND PREOPERATIVE DATA FOR 190 CASES OF SUPERIOR OBLIQUE PALSY

Sex
Male: 105
Female: 85
Age
Range: 6mo-79yr
Mean: 28.8+/-22.2 yr
Mean for congenital group: 24.1+/-21.1 yr
Mean for acquired group: 40.9+/-20.5 yr
Refraction
Mean: -0.49+/-3.04 diopters
Visual Acuity
Mean: 20/25
Median: 20/20
Congenital/acquired
Congenital: 137
Acquired: 53
Origin: Trauma 29
Iatrogenic 12
Vascular 7
Tumor 5
Knapp Classifications
Class I: 28
Class II: 13
Class III: 65
Class IV: 53
Glass V: 5
Class VI: 19
Class VII: 1
Class VIII: 6*
Laterality
Right: 92
Left: 79
Bilateral: 19
Facial asymmetry
Present: 56 51 congenital 5 acquired
Absent: 69 40 congenital 29 acquired
Unknown: 65 46 congenital 19 acquired
Abnormal head posture:
Right tilt: 55
Left tilt: 70
Others (eg, head turn, chin down): 10
No abnormal head posture: 39
Unknown: 16
Forced duction tests
Tests performed: 161
Tendon laxity: 95 83 congenital
12 acquired
No tendon laxity: 66 37 congenital
29 acquired

*Type VIII = comitant vertical deviation

Table 15
Patient demographics and preoperative data for 190 cases of superior oblique palsy.
From Helveston EM, et al. Surgical treatment of superior oblique palsy. Transactions of the American Ophthalmological
Society, Vol. XCIV, 1996, pp. 315-334. Used with permission.

158
Diagnostic categories & classification of strabismus

Table 16
From Helveston EM, et al. Surgical treatment of superior oblique palsy. Transactions of the American Ophthalmological
Society, Vol. XCIV, 1996, pp. 315-334. Used with permission.

159
Section 3

Chapter 6: Mechanics of surgery

Chapter 7: Recession of a rectus muscle

Chapter 8: Resection of a rectus muscle

Chapter 9: Surgery of the obliques

Chapter 10: Marginal myotomy: technique and


considerations

Chapter 11: Faden operation (posterior fixation suture)

Chapter 12: Adjustable sutures: techniques for


restriction

Chapter 13: Muscle transposition procedures

Chapter 14: Botox (Botulinum A toxin)


6
The mechanics of surgery

Techniques of exposure
Conjunctival incision
Overview
The conjunctival incision for strabismus surgery of these steps must be accomplished before attempt-
has two main requirements: 1) it should provide ade- ing to engage the extraocular muscle on a hook. Once
quate exposure to the muscle(s) to be operated on, sclera has been identified, the tip of the muscle hook
and 2) it should avoid excessive scarring and leave slightly indents sclera at the muscle border and is then
the conjunctiva in the palpebral opening white and passed gently behind the muscle insertion, or in the
smooth after healing has taken place. A technique for case of the inferior oblique, behind the muscle's belly.
incising the conjunctiva satisfying the first require- Any impedance of the passage of the hook behind the
ment was devised by Swan who described doing muscle insertion suggests that the hook is in the
extraocular muscle surgery beneath Tenon's capsule. wrong plane. Clean engagement of the extraocular
With this technique, conjunctiva and anterior Tenon's muscle on the muscle hook is necessary for the start
capsule are incised isolating the muscle in the plane of successful strabismus surgery
of posterior Tenon's capsule. This tissue layer makes
up the intermuscular membrane and the muscle's cap- Start of the surgical procedure
sule. Posterior Tenon's capsule is incised separately to Surgery on the extraocular muscles starts with
expose sclera. This provides access to the muscle in the placement of a lid speculum between the lids for
a physiologic "compartment" lying between anterior exposure to the front of the eye. A light, adjustable
Tenon's capsule and sclera. Swans contribution was solid bladed speculum is ideal. A standard adult size
significant because it introduced a logical approach to is used for adults and a pediatric size for children (see
the tissue planes around the rectus muscles. The Chapter 2). With the speculum in place, it is now
drawback is that the Swan incision is made over the possible to see the insertion of the rectus muscles
muscle's insertion and thereby in the palpebral open- through the conjunctiva in most cases. These appear
ing where it could heal as a raised, reddened ridge. beneath the conjunctival surface as a subtle elevation
The techniques currently used for conjunctival slightly darker than the surround. Rotation of the eye
incision like that of Swan adhere to the principle of at this point enhances the view of these muscles and
operating beneath anterior Tenon's capsule, but they also in some cases the anterior ciliary vessels further
differ in location of the initial incision through con- delineating the rectus muscles. Seeing these muscle
junctiva and in the degree of exposure of the muscle. insertions aids in orientation for the placement of the
Techniques achieving suitable exposure of the incision, especially when the eye has undergone
extraocular muscles include the following: torsion as it sometimes does with general anesthesia.
1. Transconjunctival incision in the cul-de-sac Next, it is important to perform forced or passive
(Parks) ductions in abduction, adduction, elevation and
2. Limbal incision in the palpebral opening depression testing for restrictions. The superior
3. Retropalpebral transconjunctival incision oblique traction test is done if there is any question
4. Superior cuffed limbal incision about laxity of the superior oblique tendon (see page
All of these techniques have in common an incision 97).
through conjunctiva, anterior Tenon's capsule, and
posterior Tenon's capsule exposing bare sclera. Each

163
Chapter 6

Traction sutures Choice of incision


At this time traction sutures, usually 4-0 silk, are Limbal incision
placed if that is the surgeons choice. They are put in
The limbal incision provides the best exposure to
episclera at the 6 and 12 o'clock limbus for surgery on
any of the extraocular muscles but especially the rec-
the horizontal recti and at the 3 and 9 o'clock posi-
tus muscles (Figure 3A). This incision is easy to
tions for surgery on the vertical recti (Figure 1). A
make and manage while working on the muscle. The
locking forceps, preferably curved, can be placed at
disadvantages of this incision are: 1) it requires care-
the limbus at the appropriate position to achieve
ful closure, 2) some bleeding can occur from episcle-
retraction and stabilization for surgery on any of the
ral vessels, and 3) a ridge can be seen at the limbus
muscles (Figure 2).
postoperatively if tissue edges have not been closed

A B

Figure 1
A Four-0 black silk sutures are placed in episclera at the B Four-0 black silk sutures are placed in episclera at the 3
12 o'clock and 6 o'clock limbus for traction to rotate the o'clock and 9 o'clock position to rotate the eye upward or
eye medially or laterally for exposure with the limbal downward for exposure with the limbal incision.
incision.

A B

Figure 2
A locking forceps is placed at the limbus of the right eye grasping conjunctiva and episclera to rotate the eye:
A down and in to expose the superior temporal quadrant; C up and out to expose the inferior nasal quadrant prior
B up and in to expose the inferior temporal quadrant; to the cul-de-sac incision to expose the medial rectus.

164
Mechanics of surgery

A B

Figure 3
A The locations of the limbal incision for exposure of each C Locations of the conjunctival incision for exposure of the
of the rectus muscles. oblique muscles: 1) inferior oblique, 2) superior oblique
B The locations of the cul-de-sac incision. The most useful (trochlea) nasal to the superior rectus, 3) superior oblique
one is in the inferior nasal quadrant for exposure of the tendon at the insertion.
medial rectus.

properly. While these disadvantages may sound over the muscle. Disadvantages of the cul-de-sac
daunting, they can be managed easily with sufficient incision are the following: 1) it is more difficult to
care on the part of the surgeon. Those who routinely perform; 2) exposure is less than with other incisions
use the limbal incision can manage the bleeding when especially the limbal incision. The view with the cul-
it occurs and tend to become skilled at conjunctival de-sac incision has been described as peek a boo; 3)
closure. Above all, these surgeons appreciate the there is no opportunity for recession of the conjuncti-
excellent exposure leading to what some believe is va; 4) fragile conjunctiva such as is present in older*
more accurate surgery. The limbal incision has the people can tear as the incision is stretched over the
advantage of making postoperative handling of an muscle's insertion; 5) postoperative handling of an
adjustable suture much easier. This incision is also adjustable suture can be more difficult.
important in that it allows for removal of scarred and After listing all of these disadvantages, it
unsightly conjunctiva in some cases of reoperation, should be stated clearly that the cul-de-sac incision is
and most important the limbal incision allows for ideally suited for use with recession of the medial rec-
recession of tight, restrictive conjunctiva, with or tus muscle in young children. The thick Tenon's cap-
without scarring, in cases of strabismus influenced by sule and healthy conjunctiva in the young stretch
these mechanical factors. readily over the muscle insertion providing good
Cul-de-sac incision exposure for surgery. At the conclusion of the proce-
dure, the young firm tissue slides behind the lid and
The cul-de-sac incision devised by Parks has
remains hidden completely if the procedure has been
the advantage of being hidden behind the lid. (Figure
done properly. Observing such a patient immediately
3B) In addition, it usually requires no suture for clo-
after surgery it may be difficult to detect that surgery
sure at least inferiorly where it is used most. The
has been done. This appearance changes slightly a
patient may be more comfortable in the immediate
few hours later when some swelling usually becomes
postoperative period if no excessive swelling occurs

* In this case, older can refer to patients as young as late teens or early twenties.

165
Chapter 6

evident. Although the cul-de-sac incision can be used Unique characteristics of each
to expose the lateral rectus and is also used superior- extraocular muscle in terms of initial
ly, the usefulness in these locations is far less than for exposure after the conjunctival
exposure of the medial rectus, especially in the incision
young.
Conjunctival incision for exposing oblique muscles Each of the rectus muscles has its own special
The conjunctival incision to expose the inferi- characteristics or personality. The surgeon should
or oblique is made in the middle of the inferior tem- have knowledge of this in advance for the best chance
poral quadrant about 8 mm from the limbus. It is of success at any surgical undertaking.
essential that this initial incision be posterior to the Medial rectus
line of insertion of posterior Tenon's capsule that
Of the rectus muscles, the medial rectus inserts
describes the spiral of Tillaux. An incision so placed
closest to the limbus. In the esotropic patient this dis-
goes through conjunctiva, anterior Tenon's capsule,
tance can vary from 3.0 to 6.0 mm with the stated nor-
and posterior Tenon's capsule (intermuscular mem-
mal being 5.5 mm. This variation in insertion site,
brane) exposing bare sclera after which the inferior
along with the fact that there seems to be no relation-
oblique can be seen as it passes in posterior Tenon's
ship between the distance of the insertion from the
capsule.
limbus and the angle of esotropia in esotropic
A similar conjunctival incision can be made
patients, is a reason for measuring medial rectus
just medial to or just lateral to the insertion of the
recession from the limbus rather than the original
superior rectus, but also posterior to the insertion of
insertion. The medial rectus has no other muscle or
posterior Tenon's capsule to expose the superior
orbital fascial structure associated with it other than
oblique medial to the superior rectus, near the
the intermuscular membrane to stop its retraction
trochlea, or at the insertion of the superior oblique
back into the orbit when it is detached. For this rea-
(Figure 3C).
son, the medial rectus is the most likely of any of the
Conjunctival incision and reoperation rectus muscles to fall back into the orbit if a suture
Reoperation can be done after either a limbal or breaks causing what is referred to as a slipped or
a cul-de-sac incision. The ease of re-operation in lost muscle.
either case depends more on the care exercised by the When looking at the orbital surface of the
original surgeon and the particular nature of healing medial rectus muscle, the origin of anterior Tenon's
in the individual than on the type of incision. capsule can be seen as it joins the medial rectus cap-
sule. Just outside of anterior Tenon's capsule, toward
the medial orbital wall, is the medial rectus pulley. If
a hole is made in the undersurface of anterior Tenon's
capsule at this point fat will prolapse. It is also possi-
ble with more dissection to disengage the medial rec-
The ideal conjunctival incision should have the tus from its pulley. Disruption of fat is to be avoided!
following characteristics: This can cause unwanted adhesions and worse.
Muscle immersed in fat after surgical intervention
1. Minimal scar in the palpebral opening can result in the muscle dissolving! Surgeons differ in
after surgery their approach to the intermuscular membrane when
2. Adequate exposure operating on the medial rectus. Minimal dissection of
3. Ease of performance the tissue seems to be the best choice in my opinion.
4. Absence of excessive adhesions In summary, surgery on the medial rectus should be
between Tenon's capsule, muscle sheath, carried out anterior or distal to the origins of anterior
and sclera Tenon's capsule with avoidance of any fat prolapse.
5. Ease of reoperation The surgery should avoid disruption of the pulleys
6. Able to allow relaxation of restrictive unless this is a stated aim as might be so with a mus-
scar tissue cle transfer procedure. Minimal dissection of inter-
7. Allows excision of excessive scar tissue muscular membrane should be carried out unless
8. Postoperative comfort there is a specific reason to do otherwise.
9. Allows for postoperative adjustment Superior rectus
when called for. This muscle is located farthest from the limbus
of the rectus muscles and has the broadest insertion.
It is, in my experience, the most difficult of the rectus
muscles to engage on the muscle hook, especially
when introducing the hook from the temporal side. It
is common to unintentionally split this muscle inser-

166
Mechanics of surgery

tion. When attempting to hook the muscle from lower lid retraction. Others place a suture in this tis-
either side, especially temporally, be certain to identi- sue tying it forward on the globe to keep Lockwood's
fy bare sclera posterior to the insertion of posterior and the lower lid retractors forward and thereby
Tenon's capsule. Once engaged on the muscle hook, reducing the retraction effect on the lower lid. The
it becomes clear that fat is not likely to be encoun- unique relationship of the inferior oblique and inferi-
tered, even with intermuscular membrane dissection or rectus may contribute to destabilization of the
more than 10 mm posterior to the superior rectus inferior rectus-scleral union causing this muscle to
insertion. The superior oblique tendon is fused to the slip after it has been detached and reattached at sur-
undersurface of the superior rectus by a common gery. This occurs far more frequently with the inferi-
capsule. If this connection is not severed it should or rectus than other rectus muscles. A less important
reduce the effect of a large recession of the superior but nonetheless present occurrence is the elevation of
rectus done for treatment of dissociated vertical devi- the lower lid with narrowing of the fissure with infe-
ation. This is disputed by some who claim the supe- rior rectus resection.
rior rectus remains recessed the very large (intended) Inferior oblique
amount in spite of not being freed from the underly-
The inferior oblique is engaged in the inferior
ing superior oblique. Recession of the superior rectus
temporal quadrant through an incision made just ante-
can cause retraction of the upper lid and widening of
rior to the mid portion of the distal half of the muscle.
the palpebral fissure and, conversely, resection of the
The belly of the inferior oblique is embedded in
superior rectus can cause forward movement of the
Tenon's capsule and must be shelled out. This is not
upper lid and narrowing of the palpebral fissure.
difficult, but care must be exercised to avoid splitting
Lateral rectus the muscle. At the mid portion of the exposed mus-
The lateral rectus muscle is easy to hook and is cle a large vortex vein is seen exiting sclera and enter-
usually found, as stated in anatomy books, 6.9 mm ing the orbit passing through Tenons. This is a near-
from the limbus. On the lower border of the lateral ly 100% occurrence. Exposure of the inferior oblique
rectus and about 12 mm from the muscles insertion is helped by triangulating the opening by placing a
the anterior corner of the inferior oblique insertion is muscle hook behind both the lateral and inferior rec-
found. This is an important relationship. Surgery on tus muscles and then using a retractor to pull back the
the lateral rectus, either recession or resection, can posterior edge of the conjunctival incision with
result in inadvertent inclusion of the anterior fibers of Tenons included. Inferior oblique anatomy is quite
the inferior oblique which attach along the lower bor- reliable although there have been reports of cases of
der of the lateral rectus. This usually goes unrecog- bifid insertion.
nized at the time of surgery. The result is an unex- Superior oblique
pected postoperative hypodeviation, hyperdeviation
The superior oblique muscle is not seen during
or exodeviation or a combination of these. Simply
strabismus surgery but can be seen in some cases of
freeing this attachment at reoperation does not always
medial orbitotomy. Neither is the trochlea seen
cure the problem. It is best to avoid the complication
except for the cuff of tissue where the superior
by making sure that the inferior oblique is not includ-
oblique tendon exits. It is the 30 mm tendon of the
ed.
superior oblique that is dealt with during strabismus
Inferior rectus surgery. This tendon can be extremely variable in its
This muscle is very easy to engage on a hook site of insertion, tension, and even presence! Absence
and is usually found where it should be, 6.5 mm from of the superior oblique tendon, while not common, is
the limbus. Overlying the inferior rectus about 10 the most frequently noted absent extraocular muscle
mm behind the insertion is a thick mass of fascia (tendon). Most cases where the superior oblique ten-
which comprises Lockwood's ligament with the infe- don is encountered in surgery are cases of superior
rior oblique included. This tissue is also connected to oblique underaction or palsy. A superior oblique trac-
the lower lid retractors. In addition at one or some- tion test done before an incision is made will give
times both inferior rectus borders about 10 mm from strong evidence of a tendon anomaly. Since there is
the insertion are found large vortex veins lying on the chance of significant variation with the superior
sclera. These may be seen for several millimeters on oblique either contributing to underaction as in supe-
sclera before going through Tenon's capsule and into rior oblique palsy or rigidity as in Brown syndrome,
the orbit. The relationship of the inferior rectus with good exposure is required. For this reason it is a good
Lockwood's is especially important when dealing idea to consider a superior limbal incision to achieve
with thyroid ophthalmopathy patients requiring large a thorough look at the superior oblique tendon. An
inferior rectus recession. Some surgeons dissect even better idea is to do the cuffed superior limbal
intermuscular membrane well posterior to incision which is described on page 176. For success-
Lockwooods, even encountering fat, in order to ful superior oblique tendon surgery a good rule is to
achieve a large inferior rectus recession with minimal obtain good exposure and to expect the unexpected.

167
Chapter 6

Parks cul-de-sac incision


The cul-de-sac incision of Parks is carried out and the muscle sheath, is used to retract anterior
behind the usual position of either the upper or the Tenon's capsule and the conjunctiva upward (superi-
lower lid. This procedure may be accomplished orly), exposing the tip of the first muscle hook, which
medially or laterally, superiorly or inferiorly. The is now seen at the superior border of the muscle. A
illustrations show the incision being made inferiorly snip incision through the intermuscular membrane
and nasally in preparation for surgery on the right with scissors may be required to fenestrate the inter-
medial rectus muscle viewed with the patient upright. muscular membrane, exposing the tip of the Green's
The rationale for this approach is that the space (or equivalent) muscle hook. The intermuscular
beneath posterior Tenon's capsule provides access to membrane is dissected from the muscle borders
the rectus muscles posterior to the line of rectus mus- according to the surgeon's preference. With the mus-
cle insertion (spiral of Tillaux) for 360 degrees. Shift cle exposed, sutures may be placed for a recession
of the incision which is behind the lids to over the procedure. If a resection is planned, an additional
muscles insertion is possible because the conjunctiva muscle hook or muscle clamp must be inserted to
and anterior Tenon's capsule are relatively mobile tis- expose the tendon and muscle posteriorly. It is cus-
sue planes. At the conclusion of an eye muscle oper- tomary to use one double-arm suture when carrying
ation done with the cul-de-sac incision, the incised out recession of a rectus muscle using the cul-de-sac
tissue should slide back completely behind the lid. incision, although two single-arm sutures can be
This procedure is designed to offer an improved used. After suture placement for recession, the mus-
appearance. Drawbacks of the cul-de-sac incision cle should be severed from the globe. It is essential
include inability to recess the conjunctiva and anteri- to use two fixation forceps, preferably the curved
or Tenon's capsule, limited exposure of the extraocu- self-locking type, at the corners of the insertion to sta-
lar muscles, and greater difficulty. However, in bilize the globe and keep the incision centered over
instances where good assistance is available and the operative site. It is useful to place the first lock-
when it is not necessary to recess the conjunctiva, this ing forceps on the stump of the insertion when half of
procedure can be performed effectively. The surgeon the muscle insertion is cut free. This allows the assis-
must compare these advantages with the greater ver- tant to stabilize the globe when the muscle is cut
satility of the limbal incision along with greater ease entirely free. At the conclusion of the procedure, the
of performance. conjunctiva and anterior Tenon's capsule are allowed
The cul-de-sac incision for medial rectus sur- to relax and slide back to the original incision site
gery is made approximately 4 mm below the limbus behind the lid. When performed properly, this proce-
(Figure 4). It extends 8 mm medially from the junc- dure results in a very benign appearance of the eye in
tion of the middle and medial thirds of the cornea, the immediate postoperative period because the lower
stopping just short of the base of the plica. A snip lid hides the incision.
incision is made through the conjunctiva and anterior An inferior incision may be closed with inter-
Tenon's capsule. A second snip incision through rupted, absorbable sutures or it may be left unsutured.
intermuscular membrane exposes bare sclera. A A superior incision is usually closed because of the
small segment of anterior and posterior Tenon's cap- possibility of prolapsed Tenon's capsule hanging
sule may be excised at this point. The tip of a large down in the palpebral space. The cul-de-sac incision
muscle hook is placed on bare sclera and the hook is does not allow for conjunctival recession but can be
guided upward along bare sclera until it is beneath the used for reoperations where conjunctival recession is
medial rectus muscle or the rectus muscle intended. not indicated. In infants and young children with
The hook is then drawn toward the limbus to engage healthy conjunctiva and Tenons and where the con-
the muscle at its insertion. A large Green's hook with junctiva is not restricted, I have used the cul-de-sac
a 3 mm tip at right angles at the toe of the hook may incision routinely. I have also used this incision for
be placed behind the rectus insertion to keep it from re-recession of the medial rectus in both adults and
slipping off. A second large muscle hook is placed children. It has been suggested that by leaving the
beneath anterior Tenon's capsule and is guided supe- perilimbal conjunctival circulation undisturbed, the
riorly over the original hook. The second hook is cul-de-sac incision could reduce the incidence and
then moved back and forth over the first hook to free severity of anterior segment ischemia after rectus
the fine attachments between anterior Tenon's capsule muscle surgery. A diffuse but benign subconjunctival
and the muscle sheath. During this maneuver the hemorrhage occurs in about 10% of cases after the
muscle, in its sheath, lies between the two hooks. cul-de-sac incision.
The second muscle hook, having loosened the
fascial connection between anterior Tenon's capsule

168
Mechanics of surgery

D E

F G

Figure 4
A The site of the inferior cul-de-sac incision for approach- E The initial hook may be replaced with a Green hook that
ing the right medial rectus muscle has a prominent tip. Any muscle hook with a knob at the
B The conjunctiva is tented and scissors cut down through tip is suitable for this maneuver.
the conjunctiva, anterior, and posterior Tenons to bare F A second hook is placed in the incision but on top of the
sclera. muscle. It is moved back and forth to free the muscle in
C The initial incision can include only conjunctiva. With for- its capsule from overlying anterior Tenons capsule. This
ceps grasping anterior and posterior Tenons these tis- motion should extend several millimeters anterior to the
sues are tented up and excised. muscle insertion.
D A muscle hook enters the incision and with its tip slightly G The muscle hook that was used to separate the muscle
indenting sclera the hook passes beneath the rectus from anterior Tenons capsule is then used to pull the
muscle. conjunctival incision over the tip of the muscle hook that
is engaging the muscle.
continued.

169
Chapter 6

H I

J K

Figure 4, contd
H A small snip through intermuscular membrane exposes J Putting a second hook under the muscle provides better
the tip. exposure.
I With the muscle so exposed and after sufficient dissec- K The muscle is completely detached after curved locking
tion of the intermuscular membrane at the border of the forceps are placed on the stump, the first when half of
muscle, sutures are placed. If a resection is to be per- the muscle is freed and finally the second just before the
formed, a second hook under the muscle exposes suffi- last fibers are cut.
cient muscle tissue to allow placement of sutures for L The location of the incision when the eye and lids are in
resection. the physiologic state.

170
Mechanics of surgery

Limbal incision
The limbal incision in the conjunctiva for sur- the palpebral opening. If this incision is not closed
gical exposure of the rectus muscles is probably the very carefully with a smooth approximation of the
easiest to perform and the most versatile of the expo- conjunctiva - anterior Tenon's capsule layer with the
sure techniques for strabismus surgery. Wide expo- limbus, an unsightly ridge could result. This ridge
sure of the muscle and adjacent sclera with the inci- can also cause inefficient wetting of the peripheral
sion placed directly over the area of surgical activity cornea resulting in delle formation. The neophyte
makes suture placement in the muscle and needle strabismus surgeon should, in my opinion, learn the
placement in the sclera easier than with the cul-de-sac limbal incision first.
incision. When muscle transfer or insertion shift is The site of the limbal incision for medial rectus
done up or down, this wide exposure is especially surgery is centered at the insertion of the rectus mus-
beneficial. The relaxing incisions may be extended cle and extends 2 to 3 clock hour positions. The
10 mm or more without penalty because the addition- fusion of conjunctiva and anterior Tenon's capsule is
al length of these incisions is hidden behind the lids. grasped with fine-toothed forceps and tented up, and
The limbal incision also enables the surgeon to carry subanterior Tenon's capsule is entered with a No. 15
out conjunctival recession or conjunctival excision of Bard-Parker blade (Figure 5) scissors may also be
scarred tissue when necessary and also debulking of used as shown (Figure 5B). This blade incision is a
anterior Tenon's capsule in selected cases. A draw- puncture, not a dissection. The blade should not dig
back of this procedure is that an incision is made in into the episclera or sclera. Scissors should be used

A B

C
E

Figure 5
A The conjunctiva at the limbus is tented and a sharp blade C First, the radial incision is made,
passes beneath anterior Tenons capsule. D then the limbal incision,
B Scissors may be used to initiate the limbal incision. The E and then the second radial incision.
conjunctiva is tented and the scissor tips cut down along
the line of the radial incision through conjunctiva and
anterior Tenons capsule.

171
Chapter 6

to extend one relaxing incision. Scissors are then other border. Care should be exercised to avoid forc-
used to complete the limbal peritomy and then the ing the hook past or through an incompletely dissect-
second radial relaxing incision is made. These relax- ed plane. The intermuscular membrane is dissected
ing incisions are carried to but not through the plica from the borders of the muscle and the attachments
when the incision is made medially. Scissors and between the muscle sheath and the undersurface of
sharp dissection are used to sever the attachments anterior Tenon's capsule are dissected according to
between the muscle sheath and the undersurface of the surgeon's preference. Sutures are placed and the
anterior Tenon's capsule. Bare sclera is exposed at muscle is recessed as shown (Figure 7) or the intend-
one or both borders of the muscle's insertion by pierc- ed procedure is done. The limbal flap is closed at the
ing the intermuscular membrane at each edge of the apices with interrupted 8-0 absorbable sutures. An
muscle's insertion (Figure 6). It is imperative that additional suture may be placed in each of the radial
bare sclera be identified during this maneuver to incisions.
allow smooth passage of the muscle hook behind the The limbal flap may be recessed 5 mm using
insertion of the rectus muscle. Dissection of the mus- three sutures. Two of the sutures join the tips of the
cle capsule itself should be avoided and the muscle flap at the base of the relaxing wing incisions, and the
should remain in its capsule. Failure to maintain this third suture secures the center of the flap to the super-
technique produces unnecessary bleeding. ficial sclera or the muscle stump. The knot securing
When bare sclera is identified at each border of the conjunctival flap at the limbus may be buried by
the muscle, a muscle hook is passed easily behind the passing the needle from beneath the conjunctiva at
insertion. It is also acceptable to pass the muscle the limbus, and then from the conjunctival surface on
hook behind the insertion after exposing bare sclera at the flap, and finally tying the knot down tightly so
one border and cut down on the tip of the hook at the that it slides under conjunctiva.

B
C

Figure 6
A The conjunctiva-anterior Tenons flap is retracted and B A muscle hook is passed behind the muscle insertion.
scissors are used to penetrate posterior Tenons capsule C Shown here at surgery
exposing bare sclera at each muscle border.

172
Mechanics of surgery

B C

Figure 7
A After suture placement the muscle is recessed (or the C Shown here at surgery.
intended procedure is completed) and the surgical site is D The conjunctiva may be recessed.
observed. E The sutures may be placed to bury knots for more com-
B The incision is closed with fine absorbable suture joining fort in the immediate postoperative period.
the corners at the limbus.

173
Chapter 6

Incisions for exposing the


obliques
Exposure of the oblique muscles (tendon in the at the medial aspect of the insertion of the superior
case of the superior) can be obtained effectively by rectus muscle (approximately 8 mm from the limbus)
using a transconjunctival incision behind the lids and extends through the conjunctiva, anterior Tenon's
(Figure 8). When one is making this incision, it is capsule, and intermuscular membrane for 8 mm
imperative to make it approximately 8.0 mm from the medially, concentric with the limbus. The incision for
limbus to ensure that the subposterior Tenon's space exposure of the superior oblique tendon at its inser-
will be entered. When operating on the inferior tion is begun at the lateral corner of the superior rec-
oblique, an effective technique is to hook both the lat- tus insertion (approximately 8.5 mm from the limbus)
eral and the inferior rectus muscles and retract con- and extends through the conjunctiva, anterior Tenon's
junctiva-Tenons before hooking the inferior oblique. capsule, and intermuscular membrane laterally for 6
When operating on the superior oblique, the superior mm, concentric with the limbus. The incision for
and medial rectus muscles may be hooked before exposure of the inferior oblique is made 8 mm from
hooking the superior oblique. In cases where the the limbus, is approximately 8 mm long, and is cen-
superior oblique is to be exposed prior to a strength- tered in the inferior temporal quadrant, concentric
ening procedure or in cases of Brown syndrome, I use with the limbus. The incision is carried through the
the cuffed limbal incision in order to carry out a conjunctiva, anterior Tenon's capsule, and intermus-
more orderly exploration of the superior oblique ten- cular membrane to bare sclera. The incision for a
don, which is frequently anomalous in congenital combined procedure on the lateral rectus and inferior
superior oblique palsy. oblique is a standard limbal incision that is extended
The incision for exposure of the superior inferiorly one additional clock hour.
oblique tendon medial to the superior rectus is begun

A B

C D

Figure 8
A Location of the conjunctival incision to expose the superi- C Location of the conjunctival incision to expose the inferior
or oblique medial to the superior rectus oblique
B Location of the conjunctival incision to expose the superi- D An enlarged limbal incision to expose both the lateral
or oblique tendon at its insertion rectus and inferior oblique

174
Mechanics of surgery

Obtaining improved exposure


Improved exposure can be gained by enlarging a they are regular 7 mm, large 9 mm, and extra large 11
limbal incision as needed. mm wide respectively. Each have a blade length of
A new retractor designed specifically for 15 mm.
extraocular muscle surgery is called the Barbie. This This retractor is designed to replace the bulky
was named in reference to the popular Barbie doll Desmarres (vein) retractor and the heavier ribbon or
when a scrub nurse suggested that the new retractor maleable retractors - both borrowed from general
looked like something Barbie could use to flip a ham- surgery (Figure 9).
burger. The Barbie retractor comes in three sizes and

Figure 9
A The Barbie retractor C A wide Desmarres retractor retracting Tenons and con-
B A ribbon-maleable retractor elevating the superior junctiva over a horizontal rectus muscle
oblique tendon and superior rectus

175
Chapter 6

Cuffed superior limbal incision


The superior limbal quadrant differs from about 4 to 5 clock hours centered over the superior
the other 270 degrees in that it is less distinct and is rectus. Two lateral wing incisions are made for 6-8
traversed by multiple fine blood vessels going from mm as in the standard limbal incision and surgery is
the conjunctiva to the cornea. This quirk of anatomy carried out in the usual way. The incision is closed
makes it more difficult to achieve a clean limbal inci- meticulously with several interrupted 8-0 absorbable
sion superiorly and it also leads to peripheral opacifi- sutures with the knot buried. This incision heals in
cation of the superior cornea when a standard limbal just a few days with virtually no trace of surgery hav-
incision is done. To avoid this complication the ing been done. It is possible that the molding effect
cuffed limbal incision can be made. This consists of of the upper lid as it moves over the incision line con-
a curvilinear incision in conjunctiva about two mm tributes to this healing effect (Figure 10).
above the superior limbus. The incision should be

A B

C D

Figure 10
A The initial conjunctival incision is made temporally at the C The incision is carried out in the usual manner for the lim-
10 o'clock right eye and 2 o'clock meridian left eye 2 mm bal incision exposing the superior rectus and later the
posterior from the limbus. superior oblique tendon if that is the aim.
B The incision is continued through conjunctiva and anterior D The cuffed limbal incision is closed with several interrupt-
Tenon's for 4 or 5 clock hours leaving a 2 mm cuff on the ed absorbable 8-0 sutures with the knots buried.
limbal side.

176
7
Recession of a rectus muscle

Overview
Measured retroplacement or recession is the in vertical widening of the palpebral fissure.
standard technique for weakening a rectus muscle. Excessive recession of the superior rectus can cause
However, the term weakening or reducing the effect retraction of the upper lid and a widened palpebral
of a muscle may be a misuse of terms. Instead, retro- fissure. The unique relationship of the inferior rectus
placement of a rectus muscle provides a new starting to Lockwood's ligament and the inferior oblique
place or static alignment for the eye. In this new causes the inferior rectus, after recession, to be prone
position, the muscle's attachments to both anterior to both early and late posterior migration (slippage).
and posterior Tenon's capsule, the muscle pulleys, This results in undesirable overcorrection-hyper-
and adjacent structures continue to affect both static tropia, underaction of the muscle, and lower lid pto-
and dynamic factors in eye movement. The muscle's sis.
effect on the globe is mediated through these attach- To avoid this complication, the inferior rectus
ments as well as through the muscle's scleral inser- should be securely reattached to the globe. The prob-
tion. Unless the rectus muscle is recessed excessive- lem of lower lid ptosis after inferior rectus recession
ly, placing the new insertion behind the equator along can be reduced if not completely eliminated by taking
with extensive Tenon's dissection, the muscle's action two precautions. First, the intermuscular membrane
will not be compromised significantly in its field of (posterior Tenon's capsule) dissection should be car-
action. ried back 10 to 14 mm posterior from the inferior rec-
Both saccadic velocity and generated force will tus insertion. In most cases this maneuver requires
be the same after the usual recession. Excessive careful dissection to a point several millimeters pos-
recession, either by design as in special cases or as a terior to the entrance of the vortex veins. These are
complication, will result in decreased excursion of the found on one or both borders of the inferior rectus.
globe in the field of action of the muscle. These large These tortuous dark red veins, 1 mm in diameter, are
recessions can be used when treating patients with visible lying on sclera traveling for 5 mm or more
conditions such as; fibrosis syndrome, third nerve before piercing posterior Tenon's capsule and enter-
palsy, thyroid ophthalmopathy, nystagmus, or with a ing the orbit. Vortex veins bleed briskly if cut. Do
variety of complicated reoperations, and in the case of not cut or tear them! If this happens, pressure should
a slipped or lost muscle. Underaction occurs not be applied and then the veins cauterized after bleed-
because of any change in the contractile power of the ing has slowed. Second, the attachments of
muscle, but because of the alteration of mechanics of Lockwood's ligament to the inner surface of the infe-
the muscle-Tenon's-globe relationship. A muscle rior oblique should be dissected carefully to the same
inserting behind the equator of the globe will not level as the dissection of the intermuscular mem-
exert its full effect on globe rotation on a purely brane. All of this dissection is carried out without
mechanical basis. exposing fat. Another technique for avoiding lower
lid ptosis is to mark the relationship of Lockwood's
Excessive recession ligament to the inferior rectus before dissection and
In addition to underaction, excessive recession muscle recession and then to suture Lockwood's to
of the medial rectus will produce a widened medial the inferior rectus in the same relative position after
palpebral-canthal area. Excessive recession of the recession as it was before - effectively pulling the
inferior rectus causes ptosis of the lower lid resulting lower lid forward and upward.

177
Chapter 7

Recession of the superior rectus has recently Recessions measured from?


been modified in some cases by use of excessively
large recessions to treat large-angle dissociated verti- The question, "Where should a rectus muscle
cal deviation. Although in the past it was considered recession be measured from?" has not been answered
dogma to restrict vertical rectus recession to relative- fully. Gillies and McIndoe advocated the use of axial
ly smaller amounts, such as 5 mm or less, some sur- length measurement to produce more accurately
geons now believe that excessive recession of the dosed surgery. This approach certainly makes sense
superior recti, usually via hang-back recession, is on two counts. First, a smaller axial length means a
both safe and effective. This raises the question: smaller globe, which in turn means that theoretically
Where does the superior rectus reattach when a hang- more effect is produced per millimeter of recession.
back recession is done? It has been shown clearly Second, knowing the axial length allows the surgeon
that the firm attachment of the superior oblique ten- to know where the equator of the globe is and, there-
don to the undersurface of the superior rectus theoret- fore, to recess the muscle maximally with more pre-
ically limits the extent of superior rectus retroplace- cision and still avoid excessive recession.
ment when using the hang-back technique. In order Since 1974, I have used the limbus as the ref-
to truly hang back the superior rectus muscle, it erence point for medial rectus recession. The globe
seems the surgeon should detach the superior oblique undergoes a more or less orderly enlargement with
tendon from the undersurface of the superior rectus. age in patients with refractive errors 4.00 D or less.
Even with this accomplished, the surgeon cannot be The corneal diameter also increases in a predictable
assured that the superior rectus will remain recessed way and that is why we choose the corneoscleral lim-
to the degree that the hang back implies. Hang-back bus as a reference for medial rectus recession. A
recession of as great as 15 mm from the insertion or maximum recession of 10.0 mm from the limbus is
approximately 5 mm behind the equator has been performed on eyes of infants under 6 months of age,
claimed. Because of the attachment of the superior 10.5 mm under 1 year, and 11.5 mm on eyes of
rectus to underlying superior oblique this amount of patients over 1 year (Figure 1). In addition to provid-
recession can be seen with the eye rotated downward. ing a standard of measurement related to globe size
However, when the eye rotates back to the primary and, therefore, adhering to geometric factors, meas-
position, the superior rectus moves closer to the inser- urement from the limbus is a convenient way to avoid
tion along with the attached superior oblique tendon. using the variable medial rectus insertion site as the
Recessions of the superior rectus muscle by measured reference for recession measurement. We found the
recession or by the hang-back technique that exceeds medial rectus insertion site to vary from 3.5 to 6.0
5 or 6 mm could result in limited elevation. But since mm with an average of 4.4 mm in a group of esotrop-
this surgery is usually done for .dissociated vertical ic patients. In these patients there was no relationship
deviation (DVD) in patients who have effective sup- between the medial rectus insertion site and the angle
pression the resulting incomitance does not lead to of strabismus.
diplopia and the limitation of supraduction is not sig- Our initial motivation for using the limbus was
nificant. that an all too common result of surgery for infantile
Recession of the lateral rectus is more or less esotropia in the 1960's and 1970's was undercorrec-
limited by the insertion of the inferior oblique found tion. To reduce this unacceptably high percentage,
12 mm from the lateral rectus insertion. However, in which resulted in nearly 50% of surgically treated
hang-back recession, or in excessive recessions of esotropia patients requiring a second operation after
the horizontal recti carried out to treat nystagmus, this bimedial recession, surgeons gradually began increas-
limit may be exceeded with the new lateral rectus ing the amount they recessed the medial rectus while
inserted at or behind the anterior edge of the inferior still measuring from the insertion. The largest medi-
oblique insertion. For the usual strabismus surgery, al rectus recession as measured from the limbus for
the lateral rectus is recessed from a minimum of 4 to many surgeons had been 5.0 mm in the 1960's. This
5 mm to a maximum of 8 to 10 mm. Recession of the amount gradually was increased to 5.5 mm, 6.0 mm,
lateral rectus in excess of 8 to 10 mm can result in and finally 7.0 mm. By ignoring the insertion site of
some limitation of abduction. the medial rectus and using instead the limbus as a
Recession of the lateral rectus can be compli- reference, I decided to establish for my surgery a
cated by inadvertent inclusion of the inferior oblique. maximum and a minimum recession. A recession of
This complication can be reduced if not eliminated if 8.5 mm from the limbus, which was determined to be
the surgeon is certain that the inferior oblique is not the minimum, could actually be a maximum recession
brought forward when hooking the lateral rectus and if the medial rectus was inserted 3.5 mm from the
second by carefully inspecting the lateral rectus rein- limbus and if the surgeon used 5.0 mm as the maxi-
sertion site and removing any inferior oblique fibers mum medial rectus recession. The strategy of medial
or intermuscular membrane that may be pulling the rectus recession measured from the limbus has result-
inferior oblique forward. ed in fewer patients having an unintended under

178
Recession of a rectus muscle

recession and resultant undercorrection. By measur-


ing from the limbus when recessing the medial recti,
more than 80% of patients treated by me for infantile
esotropia with a bimedial recession have a residual
strabismus (almost entirely esodeviation) of less than A
10 prism diopters, and just fewer than 10% require
surgery for early postoperative undercorrection.
Does the amount of shortening of the muscle,
or rather the amount of shortening of the distance the
muscle travels from origin to the new insertion site,
dictate the postoperative effect of the muscle? Are
the sarcomeres more lax and less vigorous in their
effect on rotation of the globe? The lack of postoper-
ative change in saccadic velocity and generated mus-
cle force suggests this is not a major factor and cer-
tainly not the principal factor in altering the position B
of the eye after recession. Does the new position of
the muscle on the globe dictate the new alignment on
a purely mechanical basis? The fact that alignment
does not change in the case of a hang-back recession
between the initial alignment when the suspending
sutures are the effective insertion and the time when
the muscle attaches to the globe makes this doubtful.
The truth may be that both factors have some influ-
ence. The fact that the posterior fixation suture is Figure 1
effective in reducing movement of the globe in a mus- A Minimum medial rectus recession is 2.5 mm from the
cle's field of action certainly speaks for the impor- insertion or 8.5 mm from the limbus
tance of a recessed muscle's insertion site. The mus- B Maximum medial rectus recession less well defined
(could be 6-7 mm from insertion or 11.5 mm from the
cle length remains unchanged with the posterior fixa-
limbus).
tion suture and in most cases the primary position
remains unchanged. In addition, this procedure has
no effect on alignment in gaze in the direction oppo-
site the side of the procedure.

measure from the insertion site. This technique for


Four important points to remember when these other rectus muscles allows sufficient recession
doing rectus muscle recession are: (1) do enough amount with or without resection of the antagonist
recession to achieve the intended result; (2) do according to the patients needs. I do not encounter
not recess the muscle so far as to produce an chronic undercorrections after recession of the other
undesired result such as deficient duction, rectus muscles, as had been the case with medial rec-
widened fissure, or lower lid ptosis; (3) be con- tus recession.
sistent; and (4) make adjustments in the amount
of recession done in subsequent patients based on Medial rectus recession
your past surgical results.
Measured recession or retroplacement of the
medial rectus is the procedure of choice for weaken-
A small change in the medial rectus insertion ing this muscle in esodeviations. In certain situations,
location relative to the limbus when measured before marginal myotomy is a satisfactory and even pre-
and after detachment of the muscle has been ferred technique for weakening any of the rectus mus-
described. Although this change may be of some the- cles, but marginal myotomies should be reserved for
oretical interest, it does not represent an important specific indications (see chapter 10). A modified
clinical consideration. The medial rectus insertion recession procedure is accomplished with the hang-
site has been said to shift 0.3 mm closer to the limbus back technique. I prefer to dissect intermuscular
after detachment of the medial rectus and when the membrane a minimal amount, just enough to place
eye is abducted with forceps. This factor is another, sutures in the case of a first surgery. Reoperations are
albeit minor, factor that makes recession measure- dealt with on a case by case basis but, there is proba-
ment from the limbus logical for medial rectus reces- bly not much value in carrying the dissection any fur-
sion. For recession of the other rectus muscles, I ther in these cases.

179
Chapter 7

A minimum medial rectus recession for those


A who measure from the insertion is 2.5 mm. This is a
reliable figure and should not be violated. A medial
rectus recession of less than 2.5 mm is rarely, if ever,
justified. The resection-effect of suture placement
B and the fibrosis of healing tend to nullify any expect-
ed muscle weakening effect if a recession smaller
than 2.5 mm is performed. An exception to this is the
proposed modifying effect that is said to occur from
simply detaching and reattaching a muscle. This pro-
cedure has been suggested by Hertle done on the four
horizontal recti for damping nystagmus.
A maximum medial rectus recession measured
from the limbus had traditionally been 5.0 or 5.5 mm.
This figure is based on the fact that moving the medi-
C
al rectus farther than 5.5 mm posterior to its normal
insertion places the new insertion behind the point of
Figure 2 tangency with the globe. This point of tangency is
Dimensions related to medial rectus recession measuring
from the limbus anterior to the equator of the globe because the origin
A Corneal diameter of the medial rectus at the ligament of Zinn is medial
B Distance of equator from limbus to the anteroposterior axis of the globe. If the con-
C Variation in medial rectus insertion in patients with tracting medial rectus acts on the globe as if it were a
esotropia
string attached to a ball, no unwrapping or rotational
effect would be expected if the muscle attached
behind the point of tangency. The muscle in such an
instance would act more as a retractor than an adduc-
tor. However, because medial rectus action on the
globe is mediated through attachments to the inter-
muscular membrane (posterior Tenon's capsule and
the pulleys), certain cases requiring extra weakening
effect of the medial rectus can be treated with a reces-
sion larger than 5.5 mm, or even with a free tenotomy
in extreme cases. Many surgeons who perform medi-
al rectus recession measured from the muscle's inser-
tion exceed this 5.5 mm recession rule. They recess
the medial rectus 6 or even 7 mm or possibly more
from the original insertion. Because of variations in
the point of insertion of the medial rectus (average 4.4
mm - range 3 to 6 mm) I perform medial rectus reces-
sion using the limbus as the point of reference. These
recessions range from 8.5 mm to 11.5 mm (Figure 2).
In selected cases this upper limit of recession is
exceeded, such as in cases of nystagmus where the
medial rectus has been recessed up to 14.0 mm from
the limbus, and therefore behind the equator when the
four-muscle recession procedure is done. These
large recessions produce limitation of ductions.
Even when the medial rectus is recessed to its
functional point of tangency, attachments to the inter-
muscular membrane, which in turn attach to the globe
well anterior to the point of tangency and medial to
the globe's vertical axis, can facilitate adduction. The
lever arm is reduced, but adducting power remains.
The extent to which the intermuscular membrane is
severed at the muscle border can influence the degree
Figure 3 of weakening accomplished by a given medial rectus
Medial rectus recession with minimal dissection of inter- recession (Figure 3). An extreme example is the case
muscular membrane of a slipped or lost muscle that has had extensive free-

180
Recession of a rectus muscle

Lateral rectus recession


Measured recession or retroplacement of the
lateral rectus is the procedure of choice for weaken-
ing this muscle in exodeviations. In certain instances,
a marginal myotomy is a satisfactory and even desir-
able procedure for weakening the lateral rectus mus-
cle, but this procedure should be reserved for specif-
ic cases (see Chapter 10). A modified recession pro-
cedure is accomplished by the hang-back technique.
A minimum lateral rectus recession is 4 mm.
Less recession should not be undertaken if surgery to
weaken the lateral rectus is justified. The maximum
measured lateral rectus recession had been 7 mm in
adults and 6 mm in children. However, most sur-
geons now perform 8 mm and even up to 10 mm or
even larger recessions of the lateral rectus without
crippling the muscle's effect (Figure 5).
Large recessions of the lateral rectus may be
performed in certain cases without severely restrict-
ing motility because the muscle continues to act
through attachments to the intermuscular membrane.
The lever arm is reduced, but abducting power

Figure 4
After extensive dissection of the intermuscular membrane
the medial rectus, if it becomes detached from the globe,
can retract into the fat of the muscle cone resulting in a
lost muscle.

ing of the intermuscular membrane from the muscle B


borders. In such a case little, if any, adduction is pres-
ent postoperatively. On the other hand, free tenotomy
performed with minimal dissection of the adjacent
intermuscular membrane leaves the patient, in most
cases, with some adduction. Free tenotomy may be
an unsound procedure more because it is unpre-
dictable than because it is crippling.
Minimum dissection of the intermuscular
membrane and overlying Tenon's capsule would the-
oretically lessen recession effect and lessen the likeli- Figure 5
A Minimum lateral rectus recession, 4.0 mm
hood of a lost muscle. Maximum dissection of the B Maximum lateral rectus recession, 8.0 mm
intermuscular membrane would theoretically maxi-
mize the recession effect and could with suture failure
result in a lost muscle (Figure 4).

181
Chapter 7

remains. The extent to which the intermuscular mem-


brane is severed from the muscle border can influence
the degree of weakening accomplished by a given lat-
eral rectus recession (Figure 6). As with the medial
rectus, an extreme example is the case of a slipped or
lost muscle that has had extensive freeing of the mus-
cle borders from intermuscular membrane. In these
cases little, if any, abduction is present postoperative-
ly. On the other hand, free tenotomy, which is always
performed with minimal dissection of the adjacent
intermuscular membrane, in most cases leaves the
patient with some abduction. Free tenotomy is infre-
quently done. It is often unpredictable and can be
crippling. However, in some cases of large angle
exotropia in a previously operated patient who may Figure 6
Lateral rectus recession with minimal intermuscular mem-
have mechanical restrictions, free tenotomy or one
brane dissection
guarded by an adjustable suture may be done. This
may also be considered a type of hang-loose proce-
dure with more posterior globe-suture attachment. In
extreme cases where lateral rectus function needs to
be eliminated, the lateral rectus is detached and reat-
tached to the lateral orbital periostium.
When attempting to engage the lateral rectus
with a muscle hook, care should be taken to avoid
inadvertently including all or part of the inferior
oblique muscle at its insertion (Figure 7). This com-
plication can be avoided by making the initial sweep
of the hook from above. If the hook is passed upward
from below, it must not be thrust too deeply into the
orbit. Inclusion of the inferior oblique in lateral rec-
tus recession will, if undetected, lead to unpredictable
surgical results accompanied by restrictions in motil- Figure 7
ity. This has been called the inferior oblique inclu- Care should be exercised to avoid unintended inclusion of
sion syndrome. the inferior oblique when hooking the lateral rectus
When the lateral rectus is detached from the
globe, the muscle should be lifted and the undersur-
face and the inferior border of the lateral rectus
should be freed from the inferior oblique muscle
(Figure 8).

Superior rectus recession


A minimum recession of the superior rectus is
2.5 mm. A recession smaller than this would be inef-
fective and should not be performed. A maximum
recession of the superior rectus had been thought to
be 5 mm, although some surgeons routinely exceed
this figure placing the superior rectus at 10 mm and
up to 15 mm from the original insertion, especially in
cases of DVD (Figure 9). Large recessions of the
superior rectus muscle can cause retraction of the
upper lid leading to widening of the palpebral fissure.
The superior oblique tendon passes beneath the supe-
rior rectus approximately 5 mm posterior to the nasal Figure 8
aspect of the superior rectus insertion. A recession of It is good practice to lift the lateral rectus to confirm that the
inferior oblique is not attached to the lateral rectus or
the superior rectus greater than 5 mm would place the
included in the suture. The relationship of the lateral rectus
new insertion of the superior rectus at the superior and inferior oblique makes it unlikely that the lateral rectus
oblique tendon if the tendon were dissected free or will be lost.

182
Recession of a rectus muscle

A B

Figure 9
A Minimal superior rectus recession is 2.5 mm B Maximum superior rectus recession is not established. A
5.0 mm recession will place the new insertion anterior to
the superior oblique tendon.

would deflect the tendon posteriorly if this freeing guided just behind the superior rectus insertion. In
had not been done. spite of efforts to avoid this complication, the superi-
The superior rectus insertion can be engaged or oblique tendon may be inadvertently included with
from the medial or the lateral side (Figure 10). the hook which is intended to engage only the superi-
Careful dissection exposing bare sclera should be or rectus. If this inclusion goes unrecognized, the
completed before inserting the muscle hook. To superior oblique tendon may be reattached at the new
avoid engaging the superior oblique tendon, an inci- insertion of the recessed superior rectus (Figure 11).
sion is made to enter subposterior Tenon's space We have seen this complication at reoperation. If the
medial to the insertion of the superior rectus. The tip surgeon observes or suspects this unintended superior
of the muscle hook gently indents bare sclera and is oblique inclusion, a second muscle hook is passed

A B

Figure 10
A When identifying the entire superior rectus insertion it B The initial attempt to hook the medial rectus can be
may be necessary to pass a hook from either border and made from the medial side.
sometimes several times.

183
Chapter 7

A B

Figure 11
A Hooking the superior rectus from the nasal side can B A second hook introduced from the temporal side can
inadvertently include the superior oblique tendon. free the superior oblique tendon. The first hook is then
removed.

from the temporal border of the superior rectus after


the tip of the first hook has been rotated backward.
As the second hook is inserted, the first hook is with-
drawn. With the superior oblique tendon freed from
the hook engaging the superior rectus, another hook
may finally be placed from the medial side or from
the lateral side of the insertion. In my experience, the
superior rectus insertion is the most likely of the rec-
tus muscles to be split when attempting to engage it
on a muscle hook (Figure 12). This is more likely to
occur when hooking the muscle from the temporal
side because the insertion tends to curve posteriorly.
If this occurs, re-hooking from the other border will
allow inclusion of the total muscle (Figure 13).
Figure 12
Hooking the superior rectus from the temporal side can split Inferior rectus recession
the insertion.
A minimum recession of the inferior rectus is
2.5 mm. A recession smaller than this would proba-
bly be ineffective and should not be performed. A
maximum recession of the inferior rectus under most
circumstances is 5 mm (Figure 14). This amount is
not ordinarily exceeded because an excessively large
recession of the inferior rectus causes a pulling down
or ptosis of the lower lid resulting in a cosmetically
objectionable widening of the palpebral fissure.
These problems of the lower lid are caused by the fact
that the capsule of the inferior rectus is attached to
Lockwood's ligament and the inferior oblique muscle,
which in turn are attached to the inferior orbital sep-
tum and tarsus of the lower lid. Careful dissection of
the intermuscular membrane of the inferior rectus and
of the fascial attachments between the inferior rectus
and Lockwood's ligament can minimize the effect of
Figure 13
A second hook introduced from the nasal side can include
a large inferior rectus recession on the lower lid.
the entire tendon. The first hook is then removed. Inflammatory changes in the extraocular muscles
associated with Graves' disease have a predilection

184
Recession of a rectus muscle

for the inferior rectus. When these changes cause a


hypotropia with restriction of forced elevation of the
globe, the usual maximum inferior rectus recession
often must be exceeded to obtain adequate elevation
of the bound down globe. In such a case, inferior
A rectus recession with an adjustable suture may be
done. However, adjustable inferior rectus recession
may be complicated by early or late slippage of the
inferior rectus with widened fissure, ptosis of the
lower lid, and deficient depression (see page 403).
When dissecting the inferior rectus in cases
with or without restriction, care should be taken to
avoid cutting the vortex veins that lie at each border
of the muscle between 8 and 12 mm behind the inser-
tion (Figure 15). Also, Lockwood's ligament should
be dissected carefully with small scissor snips under
direct vision for a distance of 12 mm or slightly more
posterior to the insertion. This careful technique
reduces bleeding and the likelihood of lower lid
retraction with recession or lower lid advancement
with resection.
B
Lockwood's ligament encasing the inferior
oblique has a firm attachment to the inferior orbital
septum and the lower lid tarsus (Figure 16). The dis-
tance between Lockwood's ligament and the lower lid

Figure 14
A A minimum inferior rectus recession of 2.5 mm
B A maximum inferior rectus recession of 5.0 mm. This
number is exceeded in cases of restriction and in special
circumstances.

Figure 16
The connections between the inferior rectus - inferior
oblique - Lockwoods ligament and the lower lid cause the
Figure 15 structures to move together.
Vortex veins are seen on both borders of the inferior rectus A Normal
when dissection is carried back 10 mm or more. B Lower lid ptosis after inferior rectus recession without
advancement of Lockwoods.

185
Chapter 7

border tends to remain constant. As the inferior rec- Barbie or other suitable retractor is used to provide
tus is recessed and Lockwood's ligament follows the adequate exposure. When the muscle is properly
muscle, the lower lid tends to drop lower producing exposed, it is stabilized with a muscle hook. The bor-
lower lid ptosis and widening of the palpebral fissure. der of the rectus muscle is elevated with a small hook
This problem can be avoided or at least reduced if to facilitate passage of the needle between the large
Lockwood's ligament is brought forward and sutured hook stabilizing the muscle and the small hook. The
to the inferior rectus so that it is the same distance needle passes through the tendon avoiding the anteri-
from the original insertion of the inferior rectus as or ciliary vessels. The anterior ciliary vessels should
before recession (Figure 17). Lockwood's ligament is not be severed by the needle but should be included
sutured to the surface of the inferior rectus with 6-0 in the suture (Figure 18).
absorbable suture at the same distance from the orig- After passing through the tendon, the needle is
inal insertion as it was preoperatively. brought again through the tendon including the ante-
rior ciliary vessels. The suture is then locked*
Rectus muscle recession (Figure 19). This technique for suture placement
technique
The standard technique for recession of each of
the rectus muscles is the same. Differences in maxi-
mum and minimum amounts and management of the
intermuscular membrane and check ligaments were
discussed previously. Because of the strength and
uniformity of synthetic absorbable suture, I prefer to A
recess a muscle using one double-arm suture.
However, two single-arm sutures may be used.
The rectus muscle is exposed by carefully
incising the intermuscular membrane and posterior
Tenon's capsule at 2 or 3 mm from the muscle border
with sharp dissection. Anterior Tenon's capsule aris-
es from the outer surface of the muscle sheath and is
outside of posterior Tenon's capsule. Anterior
Tenon's capsule is dissected with scissors from the
outer surface of the muscle until suitable exposure is
obtained. This varies according to each muscle. No
fat should be exposed. Large vessels bridging from B
the muscle capsule surface to the undersurface of
anterior Tenon's capsule may be simultaneously sev-
ered and sealed with cautery (see chapter 3). A

Figure 18
A Exposure of the rectus muscle insertion includes freeing
Figure 17 the insertion and proximal muscle borders sufficiently to
To assist in reducing lower lid ptosis when recessing the place the sutures.
inferior rectus the tissues around Lockwoods ligament are B A small hook elevates the muscle border to facilitate
pulled anteriorly and sutured to the inferior rectus at the placement of the needle through the muscle.
same distance from the inferior rectus insertion as in the C The needle is passed through the tendon avoiding the
preoperative state. anterior ciliary arteries.

* Mims has made specific recommendations for placing a secure locking bite, which is actually a true knot, at the rectus muscle border.

186
Recession of a rectus muscle

A B C

Figure 19
A If the sutures are passed as shown a true knot is C The muscle is detached.
formed.
B The anterior ciliary arteries are ligated the hook is aimed
back toward the orbit (not shown).

behind the first hook reduces the likelihood of inad- measuring medial rectus recessions and continue to
vertently cutting the suture when detaching the mus- use it. When comparing measurement from the lim-
cle and reduces bleeding by ligating the anterior cil- bus with measuring from the insertion, the distance
iary vessels. The resection effect is negligible in my between the insertion and the limbus should be added
opinion. After the sutures have been pulled posteri- to the intended amount of recession. When measured
orly from the insertion, the muscle is cut off flush from the limbus, a traditional 4 mm recession of the
with the sclera using scissors and taking small snips. medial rectus would indicate that the new insertion
Several footplates may need to be severed before the should be 9.5 mm from the limbus, assuming that the
muscle retracts freely. The muscle hook is now medial rectus insertion is 5.5 mm from the limbus.
behind the suture line for cutting the muscle free, Clinical experience has shown that the average medi-
whereas it was in front of or closer to the insertion al rectus insertion in esotropia is 4.4 mm with a range
when the sutures were placed. of 3 to 6 mm. Measuring from the limbus opposite
A caliper or scleral ruler is used to measure the the corner of the insertion advances the muscle a bit
amount of recession. Measurements may be taken (decreasing the recession) and measuring a chord pro-
from the original insertion or the limbus. I prefer to duces excess recession. These factors are offsetting
measure most medial rectus recessions from the lim- (Figure 20).
bus. The surgeon should decide on one method of

B C

Figure 20
A A caliper measures from the limbus or the original inser- C The scleral ruler measures the true distance over the
tion. scleral surface while calipers measure the chord.
B A scleral ruler modified from William Scott is also useful
for this measurement.

187
Chapter 7

B C

Figure 21
A A passage of the needle through sclera. C The recessed muscle is ideally parallel to the old inser-
B The scleral tunnel should be 1.5 mm or more. tion (or nearly so).

The tip of the caliper or scleral ruler may be lized with a utility forceps, which may be of a lock-
used to make a dimple in the sclera. This provides a ing variety, grasping the insertion stump opposite the
point indicated by a blue mark which is the uvea site of needle placement. Care should be taken to
showing through the sclera. This dimple can be used space the two suture bites in the sclera approximately
as a means of engaging scleral tissue with the needle 10 mm apart and equidistant from the limbus or inser-
tip. The thin, spatulated needle displaces tissue and tion. This maneuver ensures that the new insertion
should be in the superficial one-third to one-half of will be parallel with the limbus, smooth, and flat. The
the scleral thickness. During experiments in the lab- sutures are tied with a surgeon's knot, and a total of
oratory, Coats and Paysse found that a scleral bite 1.5 three throws are taken. The knots should be tied care-
mm long and .2 mm deep is sufficient to secure the fully, using smooth-tying forceps, grasping the suture
muscle to sclera. This bite had a pull out strength in very close to the knot.* This approach ensures secure
excess of 200 gm. This is greater than the physiolog- knots and reduces the possibility of a broken suture.
ic muscle force exerted on this reattachment. A
longer, but not deeper scleral bite can be taken to pro- Variations in suture and
duce friction in the scleral suture tunnel. This holds needle placement
the muscle in place during suture tying. A wide variety of techniques may be used to
The needle is inserted at the caliper mark secure the suture to the tendon and then to reattach the
(Figure 21). A scleral bite of 1.5 mm or more is tendon to sclera. The overriding principles are secu-
taken, but care should be exercised to ensure that the rity, consistency, and safety. The suture must be
needle is always in sight through the scleral lamellae securely attached to the tendon to avoid slippage of
and does not perforate the sclera. The globe is stabi- the tendon-muscle. The muscle and tendon must be

* Some surgeons with smaller hands than mine prefer to tie sutures using their fingers rather than forceps.

188
Recession of a rectus muscle

securely attached to sclera to avoid pulling free from placed. The scleral bite is taken and the suture is tied.
sclera and producing muscle slippage. Finally, the The crossed swords technique of Parks may also be
needle track in sclera must be deep enough and long used. The first needle is left in the long scleral tunnel
enough to secure the tendon-muscle to sclera without which angles toward the insertion while the second
going too deeply and entering the eye by passing needle is placed in the sclera, crossing the first. Both
through choroid and retina into the vitreous. needles are advanced carefully pulling the first suture
As a variation of the double-arm suture tech- through only when the round part of the other needle
nique, a single suture may be used, taking a bite into remains in the track. This maneuver prevents the sec-
the central tendon and then tied. Additional bites then ond needle from cutting the first suture while in its
are taken at the muscle borders and locked loops are scleral track (Figure 22).

Figure 22
A A bite secured with a knot can be taken at mid-muscle B The properly recessed muscle with a double arm suture.
and sutures brought out the borders and locked for C Needles can be placed in the crossed swords technique
added security. producing a longer scleral tunnel. This holds the muscle
in place while the suture is being tied.

189
Chapter 7

Vertical displacement of the


horizontal rectus
In cases of vertically incomitant strabismus To know the proper direction for vertical dis-
(A or V pattern) without apparent overaction or placement, the surgeon needs to remember that the
underaction of the oblique muscles, vertical displace- medial rectus muscles are always shifted to the closed
ment of the rectus muscles is effective in reducing or end and the lateral rectus muscles are shifted to the
eliminating the vertical incomitance (Figure 23). The open end. This assumes the obvious that the surgeon
muscles are moved vertically to produce more or less recesses the medial rectus for esodeviations and
relative strengthening or weakening in upgaze or resects it for exodeviations and recesses the lateral
downgaze. For example, a horizontal rectus muscle rectus for exodeviations and resects it for esodevia-
that has been recessed or resected is relatively weak- tions.
ened in the field of action corresponding to the verti- In A esotropia the medial rectus muscles are
cal direction in which its insertion has been moved. A shifted upward at least 5 mm (approximately half the
resected medial rectus that has been moved down- muscle width) and no more than 10 mm (approxi-
ward has relatively less strengthening effect in mately one muscle width). It is not firmly established
downgaze and relatively more strengthening effect in that graded amounts of vertical displacement result in
upgaze. A recessed medial rectus that is moved graded amounts of A or V pattern reduction. In
downward is weakened more in downgaze and weak- this type of recession it is important to place the new
ened less in upgaze. insertion concentric with the limbus. Therefore, it is
Miller has suggested horizontal displacement probably best to use calipers or a scleral ruler to
of the vertical rectus muscles for treatment of A and measure from the limbus. In cases of A or V pat-
V. The superior rectus muscles are moved nasally to tern without strabismus in the primary position, the
close a V pattern (reduce abduction) or temporally horizontal rectus muscles may be shifted upward or
to open an A pattern (increase abduction). The infe- downward with only enough recession to offset the
rior rectus muscles are moved medially to close an resection effect of suture placement. In V pattern
A pattern (decrease abduction) or temporally to esotropia the medial rectus muscles are recessed and
open a V pattern (increase abduction). The most shifted downward. In V pattern exotropia the later-
common use of horizontal displacement of a vertical al rectus muscles are recessed and shifted upward. In
rectus muscle is the nasal shift, which is usually per- A pattern exotropia the lateral rectus muscles are
formed when recessing an inferior rectus muscle. recessed and shifted downward. Vertical shifting of
This maneuver will eliminate or reduce an unwanted the horizontal rectus may be carried out when a reces-
postoperative A pattern. sion-resection procedure is performed. In this case,
the same rules apply.

Figure 23
A For treatment of A and V the medial recti are shifted to B The medial recti are recessed and shifted up to treat an
the closed end and the lateral recti are shifted to the A esotropia.
open end. This assumes that the proper horizontal sur-
gery has been done. continued.

190
Recession of a rectus muscle

Figure 23, contd


C The medial recti are recessed and shifted down to treat E The lateral recti are recessed and shifted down to treat
V esotropia. A exotropia.
D The lateral recti are recessed and shifted up to treat V F In the left eye, the medial rectus is resected and shifted
exotropia. up and the lateral rectus is recessed and shifted down to
treat a A exotropia.

191
Chapter 7

Resection effect of suture


placement posterior to the
muscle hook
Traditionally the suture is placed as near as bility of cutting the suture when detaching the mus-
possible to the muscle's insertion during recession cle. This also ensures a more secure union of suture
before cutting the muscle from the globe to avoid the and muscle. I believe that the new point of muscle
unwanted resection effect. It is thought that this attachment on the sclera is more important than the
resection effect will nullify a certain but unspecified muscle tendon length when the recession is large and
amount of the intended recession. I place the suture the resection effect is small (Figure 24).
about 1 mm behind the insertion to avoid the possi-

A B

C D

Figure 24
A 1 mm resection effect with a posterior (safe) suture D With no (or minimal) resection effect the same point of
placement. reattachment is achieved, but the muscle is a bit longer
B With a small resection effect, the new point of insertion is or more redundant.
more important than the slight reduction in the redundant E A potential pitfall of reducing the resection effect by
muscle. placing the suture as near as possible to the distal end of
C If the suture securing the muscle before it is detached the muscle before detaching it from the globe is slippage
from the globe is placed distal to the muscle hook (closer of the tendon and muscle in the capsule. This
to the insertion), the resection effect is avoided or at complication, called a slipped muscle is responsible for
least diminished. some early and late overcorrections, particularly after
medial rectus recession for infantile esotropia.

192
Recession of a rectus muscle

Hang-back recession
The hang-back recession has been described as with the hang-back technique. In humans who had
"a simple, safe alternative to conventional recession." hang-back recession of the superior rectus, reattach-
The procedure is said to be less likely to result in scle- ment 11.5 and 12.0 mm from the insertion (as intend-
ral perforation because needles are placed through ed by the surgeon) was confirmed by x-ray study of a
relatively thicker sclera near the insertion site. stainless steel suture placed at the end of the superior
Another reported safety factor is that because the rectus. Hang-back recession of the inferior rectus is
suture placement site is more anterior, it is more likely to result in the complication of lower lid ptosis
accessible to the surgeon. Results are said to be com- and muscle slippage. The inferior rectus muscle may
parable to conventional recession when appropriate be least suitable for hang-back recession, except in
doses are used. The attachment site has been shown desperate cases as might occur in some cases of fibro-
to be about where it was intended on studies carried sis syndrome and severe thyroid ophthalmopathy
out on monkeys who had horizontal recti recessed (Figure 25).

A B

C D

Figure 25
A Hang-back recession of the lateral rectus muscle through C Sutures may be brought through the muscle stump at the
a cul-de-sac incision. 6-0 synthetic absorbable suture is width of the muscle. A limbal incision is used.
used. D The amount of hang back recession is measured for the
B The knot in the suture is pulled to bring the cut end of superior rectus.
the muscle to the muscle stump. The suture is
measured with calipers to determine the distance the
muscle is recessed from the insertion.

193
Chapter 7

Partial disinsertion Recession of a rectus muscle


Spielmann has described slanted recession of with sparing of the anterior
the rectus muscles for treatment of head tilt without arteries
oblique muscle dysfunction. This procedure involves Detaching a rectus muscle irrevocably destroys
recession of part of the insertion of the eight rectus the ciliary artery circulation carried with that muscle
muscles. Because all rectus muscles are operated on, to supply the anterior segment of the eye. As a means
great care must be exercised to preserve the anterior of detaching a rectus muscle without compromising
ciliary artery in each muscle that is not involved in anterior segment circulation, rectus muscle detach-
suture placement and partial muscle detachment. von ment with sparing of the anterior ciliary arteries has
Noorden accomplishes the torsional Kestenbaum by been described. This technique is accomplished with
moving the entire superior and inferior rectus inser- magnification supplied by the operation microscope
tion to rotate the eye in the direction of the head tilt or by loupes. Pre-placing the sutures in the muscle
(see chapter 13). In cases of strong fixation prefer- and also in sclera at the point of intended reattach-
ence, only the fixing eye need be done. DeDecker ment is recommended to avoid stretching and possi-
accomplishes the torsional Kestenbaum by recess- ble breaking of the anterior ciliary arteries when the
ing and resecting the four oblique muscles, a daunting muscle is detached.
task in most hands (Figure 26).

Figure 26
A For head tilt to the left, the nasal half of the right superior B For head tilt to the right, the temporal half of the right
rectus and the temporal half of the left superior rectus is superior rectus and the nasal half of the left superior
recessed and this procedure is continued around the rectus is recessed and this procedure is continued
globe to rotate the eye in the direction of the head tilt. around the globe.

194
Recession of a rectus muscle

In addition to a recession, muscle transfer pro- is to be recessed 5 mm, 7 to 8 mm of anterior ciliary


cedure may be carried out with sparing of the anteri- artery should be freed from the muscle. Two single-
or ciliary arteries. When muscle transfer is done even arm sutures are then placed at the muscle's edge 1 mm
more of the anterior ciliary arteries must be freed behind the insertion and separated from the intact
from the substance of the muscle to avoid excess trac- anterior ciliary arteries.
tion on these delicate vessels. The sutures are put in sclera (pre-placed) at the
After the muscle has been exposed, the anteri- intended point of recession to prevent the muscle
or ciliary arteries are lifted up very gently with a fine from retracting so far into the orbit that the fragile
blunt muscle hook or cannula. A small snip incision anterior ciliary arteries are ruptured (Figure 27).
must be made in the muscle capsule with delicate With the anterior ciliary arteries retracted away
blunt dissection parallel to the anterior ciliary artery. from the muscle and with the pre-placed recession
A fine suture is placed very gently around the sutures in place but looped away from the insertion,
anterior ciliary artery and the artery is lifted away the muscle is detached from its scleral insertion.
from the muscle. The artery should be dissected free The muscle is tied down to sclera at the intend-
from the muscle several millimeters farther than the ed point of recession. The intact anterior ciliary arter-
muscle is to be recessed. For example, if the muscle ies continue to function uninterrupted.

Figure 27
A The anterior ciliary artery is dissected from the muscle C Sutures are placed in sclera at the point of intended
substance and is lifted on a small hook or cannula. recession.
B Sutures are placed at the borders of the muscle while
the arteries are lifted. continued.

195
Chapter 7

D E

Figure 27, contd


D The muscle is cut from its scleral insertion.
E The muscle is attached to sclera, the ciliary arteries remaining intact.

Slanted recession
Slanted reattachment of a recessed horizon-
tal rectus muscle suggested by Nemet has been used
for treatment of A and V. The muscle edge that is A
to be weakened more is recessed farther back com-
pared to the other edge. This follows the principle
of selective weakening by moving the entire muscle
in the direction that you intend to weaken the mus-
cle (Figure 28).

Figure 28
A Medial rectus top edge recessed 5 mm farther for treat-
ing an A esotropia.
B Medial rectus recession with the lower edge recessed 5
mm farther back to treat a V esotropia.

196
Recession of a rectus muscle

Y split of the lateral rectus


In cases of Duane syndrome with a tight later-
al rectus co-contraction of the medial and lateral recti
can cause the eye to shoot up or down as you would
pinch a slippery pumpkin seed through your fingers.
The up and down shoot is caused by the knife edge
of the taut lateral rectus. To lessen this knife edge
effect, a Y split can be employed to effectively
broaden the lateral rectus insertion. To accomplish
this, the muscle is isolated in the usual way. It is then
split along the long axis for approximately 15 mm.
Sutures are then placed in each of the isolated muscle
halves. These muscle halves are detached and reat- Figure 29
tached to sclera with adjacent borders separated by 10 Y split of the lateral rectus can be done
mm. The Y split may be done with or without reces- with or without recession in cases of tight
sion (Figure 29). lateral rectus muscle in Duane syndrome.

197
8
Resection of a rectus muscle

Overview
Resection of an extraocular muscle is general- more often than we had suspected. In a recent series
ly classified as a strengthening procedure. But of patients undergoing reoperation of the lateral rec-
removal of all or part of a muscle's tendon with or tus for both overcorrection and undercorrection and
without inclusion of some muscle fibers merely short- after resection or recession, 38% of patients had the
ens and does not actively strengthen a muscle, at least inferior oblique included at the inferior border of the
after the initial reflex spasticity subsides. The princi- lateral rectus. Of course, this series studied only
pal benefit of a resection may be to enhance the effect those patients needing reoperation; nonetheless, this
of a recession procedure done on the antagonist mus- specific surgical complication is one we encounter
cle. Actually, if muscle fibers are removed at the time frequently in our practice.
of resection, theoretically the muscle should be weak- As an alternative to rectus muscle resection,
er! In the clinical setting, this does not seem to be the there has been a revival of the tucking procedure for
case. As I became more aware of the relationship these muscles. An advantage of tucking is that the
between passive mechanical factors and dynamic anterior ciliary circulation of the tucked muscle can
neural factors in the surgical management of strabis- remain patent. I have used this technique on the last
mus, I performed more recessions and fewer resec- rectus muscle in a patient who had had the other three
tions. rectus muscles detached.
Although resection procedures are relatively
easy to perform, they can result in more redness and Horizontal rectus resection
lumpiness of the conjunctiva, particularly in the area (medial and lateral rectus
of the medial rectus. Natural barriers to orbital fat are muscles)
also brought more anteriorly around the medial rec- The minimum amount of resection of either a
tus, promoting the possibility of unsightly fullness medial rectus or a lateral rectus muscle is 5 mm,
after resection. Nevertheless, resection of a rectus regardless of the age of the patient (Figure 1). In gen-
muscle is indicated in many strabismus cases. eral, a resection of a horizontal rectus muscle is less
In addition to conjunctival problems that occur effective in altering ocular alignment than a recession
after resection of the medial rectus, palpebral fissure of the same amount; hence the larger relative minimal
narrowing can occur after inferior rectus resection, values for horizontal rectus resection.
and some ptosis of the upper lid, which is manifested The maximum resection for a horizontal rectus
by narrowing of the palpebral fissure, after superior muscle is 8 mm for infants less than 1 year and ordi-
rectus resection. narily 10+ mm for older children and adults.
The inferior oblique may be included inadver- However, upper limit figures for resection procedures
tently during resection of the lateral rectus. This are very loosely adhered to, in contrast to the mini-
avoidable complication can cause limited elevation mum figures which tend to be strictly followed. In a
and/or depression in the involved eye, often with patient with a very large deviation and a reason to
mechanical restriction causing unexpected horizontal limit surgery to one eye, horizontal rectus resection of
and vertical strabismus. This complication occurs

199
Chapter 8

Figure 1
A Minimum dissection of a horizontal rectus muscle is 5 B Maximum resection of a horizontal rectus is 10 mm (in
mm. special cases this can be increased to 14 mm.

up to 14 mm may be performed. Some incomitance Management of the


may result in these cases, but the benefits can out-
weigh the consequences of incomitance. For exam-
intermuscular membrane in
ple, a blind eye with 90 prism diopters of exotropia horizontal rectus resection
occurring in a patient who wishes no extraocular Fifty consecutive horizontal rectus resections
muscle surgery on the seeing eye can be treated with were performed using minimum and maximum dis-
a large resection of the medial rectus and a large section techniques alternately. It was found that a
recession of the lateral rectus, perhaps combined with slightly greater effect and slightly more predictable
a marginal myotomy. This maneuver will result in resection results were obtained after maximum dis-
straighter eyes in the primary position, and, in my section of the intermuscular membrane. This finding
experience, the incomitance produced is not bother- is contrary to what was a popularly held belief that
some to the patient and is not a cosmetic defect. It is leaving as much of the intermuscular membrane as
likely that more problems are created by horizontal possible intact produces more effect with rectus mus-
resections that are too small than by those that are too cle shortening or strengthening.
large. At the conclusion of the rectus muscle resec-
tion but before the conjunctiva is closed, additional
dissection of the intermuscular membrane may be
carried out. These attachments along with overlying
tissue attachments to anterior Tenon's capsule are
freed to allow these tissues to recede normally, reduc-
ing the bulky tissue over the resected muscle and
increasing the likelihood of free ductions after sur-
gery. During this dissection, care should be exercised
to avoid entering the orbital fat space, which is just
behind Tenons capsule at the midpoint of the muscle
(Figure 2).

200
Resection of a rectus muscle

A B

C D

E F

Figure 2
A The rectus muscle in its capsule with intermuscular E Or, the muscle clamp is placed after intermuscular mem-
membrane intact. brane is dissected several mm posterior to the extent of
B Intermuscular membrane is dissected to the extent of the resection.
resection. F The new insertion is free of intermuscular membrane. Be
C The resection clamp is placed - sutures are placed sure the muscle is firmly attached to avoid a possible
according to the surgeons preference. lost muscle!
D After resection the intermuscular membrane is at the
level of the new insertion.

Resection clamp technique for


rectus muscle resection
The technique for resection of the medial and ing to the surgeon's preference. A muscle clamp is
lateral rectus is identical. Our choice for suture mate- placed across the muscle to include the amount of
rial is 6-0 braided, coated synthetic absorbable with a muscle and tendon the surgeon intends to resect. A
.203 mm wire diameter spatula needle. caliper is used to measure from the muscle hook,
The muscle is exposed and the intermuscular which is behind the insertion of the muscle, to the
membrane and check ligaments are dissected accord- anterior edge of the clamp. Adjustments to the posi-

201
Chapter 8

tion of the clamp are made if necessary. The muscle


should not be stretched at this time. Some surgeons
prefer to measure from a point just anterior to the
muscle hook behind the insertion, to a point just pos-
terior to the muscle clamp, the point where the sutures A
are eventually placed. Larger numbers will result
when this type of measurement is made, but the same
size resection will be accomplished. An important
thing to remember when measuring a resection or any
extraocular muscle strengthening or weakening pro-
cedure is that consistent technique by the surgeon is
the only way to achieve predictable results. Because
of likelihood for variations in technique, one sur-
geon's numbers do not transfer to another.

Suture placement
After the muscle clamp has been placed accord-
ing to the measured amount of the intended resection,
the tendon is severed from its insertion. A 1 mm
tendinous stump should be left at the insertion to
serve as an anchoring place for sutures. The sclera B
immediately behind the insertion of the rectus mus-
cles is only 0.3 mm thick so this tendinous stump pro-
vides a safety factor during suture placement (Figure
3).
Double-arm sutures are inserted in a backhand
manner through the tendinous insertion. At this time
care should be taken to free the under surface and
inferior border of the lateral rectus from the inferior
oblique. If this is not accomplished, the inferior
oblique could be brought forward to the new insertion
of the resected lateral rectus, causing inferior oblique
inclusion which produces a postoperative vertical and
sometimes horizontal deviation.
The sutures are carried through the muscle
behind or posterior to the resection clamp. The assis-
tant grasps the needle tip and pulls the suture through.
The two double-arm sutures are placed in a horizon- C
tal mattress fashion, first through the insertion, and
then through the muscle behind the clamp (Figure 4).
After the sutures have been placed through the
muscle, the resection clamp is loosened and moved to
the tip of the tendon. A Nugent or other suitable for-
ceps is used to hold the tip of the tendon while the
resection clamp is advanced. Traction is placed on
the muscle clamp to advance the muscle so that the
point of passage of the sutures through the muscle is Figure 3
directly over the line of the original muscle insertion. A Measuring the resection
B Cutting the muscle from the globe. Leave a 1 mm
The sutures are tied securely with a surgeon's knot. A stump.
hemostat is used to crush the tendon just anterior to C A suture is placed backhand through the stump.
the point where the sutures are tied. A battery-oper-
ated cautery is used at this point to cauterize the
crush line on the muscle to control bleeding before
cutting off the muscle-tendon to be resected. Scissors
are used to excise the excess tendon (Figure 5).

202
Resection of a rectus muscle

A B

Figure 4
A The needle passes through the muscle behind the B The process is continued with two double armed mat-
clamp. tress sutures in place.

A B

C D

Figure 5
A The muscle clamp is advanced to the end of the muscle. C The muscle is crimped just anterior to the sutures with a
B The sutures are tied securely as the resection line is hemostat.
brought over the stump. D The crimped line is cauterized.
E The excess muscle tissue is excised.

203
Chapter 8

With the excess tendon removed, the shortened producing a smooth appearance to the conjunctiva
muscle abuts the point of the original tendinous inser- overlying the resected muscle insertion. Sutures may
tion. The double horizontal mattress sutures with be placed through the stump of the muscle from the
bites several millimeters apart afford a secure union muscle side. A lap joint is produced. It is also possi-
of the resected muscle across its entire width. A cross ble to put the needles through the muscle first and
section at the point of union shows that the tendon then through the insertion producing a butt joint
stump and muscle are joined in a slightly puckered (Figure 6).
butt joint. This gradually settles over several weeks,

Figure 6
A A butt joint. C Sutures brought through the muscle and then through
B A lap joint. the stump produce a butt joint.

204
Resection of a rectus muscle

Double-arm suture technique


for rectus muscle resection
Some surgeons prefer to resect the rectus mus- excised with scissors after the muscle is crushed with
cles, either horizontal or vertical, using one double- a hemostat just behind the insertion and just in front
arm suture. This technique lacks some of the insur- of the suture line. Each arm of the suture is brought
ance factors of using two separate horizontal mat- out through the edge of the stump of the muscle's
tress sutures, but, according to those surgeons who original insertion, and the suture may be tied to itself.
prefer this technique, it is completely safe. For more security and to prevent sag of the muscle's
The muscle is exposed and, after measurement, new insertion, each suture may be brought back
the extent of tendon and muscle resection is deter- through the insertion near its center. The sutures are
mined. A 6-0 synthetic absorbable suture is used to then brought through the central portion of the mus-
split the muscle at this point from edge to edge. A cle from beneath. The suture is gently sawed to
3 mm loop is then taken slightly behind this suture bring the remaining muscle up to the original inser-
line at each muscle border and the loops are locked. tion. A surgeon's knot is tied, securing the resected
These loops should include the anterior ciliary ves- muscle in place (Figure 7).
sels. The section of the muscle to be resected is

B C

D E

Figure 7
A A single double arm suture is placed suitable for the C The sutures are tied.
intended resection. Locking bites are then placed imme- D For added security the sutures can be brought back
diately behind the suture. through the stump and muscle...
B After cutting out the section of muscle to be resected, the E ...and tied securely.
sutures are brought through the stump.

205
Chapter 8

Resection of the superior


rectus
The minimum superior rectus resection is 2.5 to ligating the anterior ciliary vessels. A hemostat is
3 mm and the maximum is 5 mm. Resection of less used to crush the tendon just anterior to the point of
than 2.5 to 3 mm is probably ineffective, and resec- suture placement. A hot tip electrocautery can be
tion of more than 5 mm causes a forward and down- used to seal off the vessels and reduce bleeding when
ward shift of the upper lid, creating ptosis. the tendon is cut. Scissors sever the tendon along the
Because of the proximity of the superior oblique crushed line just anterior to the point of suture place-
tendon to the insertion of the superior rectus, dissec- ment. The tendon is trimmed from its insertion, leav-
tion of the intermuscular membrane of the superior ing a 1 mm stump. The previously placed sutures are
rectus before resection must be performed carefully. used to reattach the tendon to the muscle stump at the
This dissection should be carried back only a mil- point of the original insertion. Very frequently a gap
limeter or so beyond the extent of the intended resec- exists in the center of the muscle. A third suture
tion. The limited room available in the area of the placed at the center of the insertion eliminates the
superior rectus makes a free suture technique for gapping and provides a more secure union of the
resection preferable to the muscle clamp technique muscle and insertion. A single double-arm suture or
used by some for horizontal rectus resection. a tandem suture may be used in place of the two sin-
After exposing the muscle, a single arm suture is gle-arm sutures according to the surgeon's preference
placed at each muscle border at the intended point of (Figure 8).
resection. These sutures are tied with a square knot

A B

C D

Figure 8
A Two single arm sutures are placed at the muscle border C The muscle is cut anterior to the sutures.
and are secured with a square knot. D The resected muscle is cut leaving a 1 mm stump.
B The muscle is crimped. continued.

206
Resection of a rectus muscle

E F

Figure 8, contd
E The sutures are tied F An optional third suture is placed at the middle of the
insertion to close any gap.

Inferior rectus resection


technique
The minimum inferior rectus resection is 2.5 to are used to expose the area of the resected muscle and
3 mm and the maximum is 5+ mm, under ordinary tendon before placing the sutures. After measurement
circumstances. However, when a very large hyperde- with calipers, two single arm sutures are placed at the
viation is present, the surgeon may in some instances borders of the muscle. After a second pass through
safely resect up to 9 mm or more of the inferior rec- the muscle border these sutures are tied with a square
tus. The inferior rectus must be dissected carefully knot. The anterior ciliary vessels should be ligated
from its attachments to Lockwood's ligament. Also, with this suture. The muscle is clamped just anterior
between 8 and 12 mm posterior to the inferior rectus to the line of suture. The tendon and muscle to be
insertion, a vortex vein pierces the sclera adjacent to resected are excised using scissors. The two sutures
the inferior rectus muscle border. Great care should are used to reattach the tendon to the original inser-
be exercised to avoid cutting a vortex vein. tion and usually a third central suture is required to
To expose more of the inferior rectus muscle prevent gapping in the center (Figure 9).
when a large resection is intended, two muscle hooks

A B

Figure 9
A A second hook exposes the inferior rectus back to B Two double arm sutures are placed at the muscle bor-
Lockwoods ligament. ders, a second loop is added and secured with a knot.
continued.

207
Chapter 8

C D

Figure 9, contd
C The muscle is crimped anterior to the sutures. E The muscle is sutured to the globe with a central suture
D The muscle is cut in front of the sutures and then at the added.
insertion leaving a 1 mm stump.

Tandem suture technique for


rectus muscle resection
A reliable technique for rectus muscle resec- 3 mm locking bites at the muscle's border. The pos-
tion is the tandem suture. With this technique, two terior suture is placed first, just behind the intended
separate double-arm sutures are woven through the point of resection. The anterior or proximal suture is
muscle's thickness and are then locked at the muscle placed at the intended point of resection. This order
edges. This procedure is identical to placing a single of suture placement cuts down on bleeding because
double-arm suture for resection except that two dou- the posterior suture ligates the anterior ciliary vessels
ble-arm sutures are used. The sutures are placed in or at least most of them. A clamp is placed across the
the muscle stump and tied. The tandem suture pro- muscle to crush it, promoting hemostasis. A hot tip
duces a very tidy resection with the resected muscle electrocautery is applied to the crush line to help
abutting the resected muscle's insertion stump. ensure hemostasis.
Doubling the suture creates a secure reattachment. After the muscle has been crushed and cauter-
Also for adjustable resections and recessions, the tan- ized at the line of intended transection, the muscle is
dem suture is effective (see chapter 12). I also use the cut. The cut end of the muscle recedes and the prox-
tandem suture technique occasionally for horizontal imal stump is held up with forceps and is cut from the
rectus recession. globe, leaving a 1 mm stump of tendon at the inser-
After the muscle has been exposed by adequate tion. The sutures are then brought through the mus-
dissection, two double-armed 6-0 Vicryl braided, cle's stump with about 1 mm separating the sutures at
coated synthetic absorbable sutures with a 0.203 mm the respective borders of the insertion. The suture
wire diameter spatula needle are woven through the ends are then tied producing a secure reapproxima-
muscle, splitting its thickness. They are locked with tion of the cuts ends of the muscle (Figure 10).

208
Resection of a rectus muscle

B C

E
D

Figure 10
A Two double arm sutures are placed about 1 mm apart C The muscle is crimped anterior to the suture.
with an added loop secured with a knot. D Cautery is placed on the crimped line
B The anterior suture is at the point of intended resection. E The muscle is cut.
continued.

209
Chapter 8

F G

Figure 10, contd


F The excised muscle is cut from sclera leaving a 1 mm G Sutures are brought through the stump...
stump. H ...and tied.

Plication (tuck) of a rectus


muscle
When a rectus muscle is to be strengthened After exposing the muscle, a double-arm suture
(shortened), an alternative method is the plication or is placed near each muscle border using a locking
tuck. This procedure has the advantage of retaining bite. The ciliary arteries are avoided. The sutures are
an undisturbed or less disturbed anterior ciliary circu- placed a distance from the insertion equal to the
lation at least compared to the standard resection intended amount of muscle shortening. A spatula or
which transects these vessels. Some say that the pli- fine muscle hook lifts the muscle about halfway
cation when performed carefully produces less post- between the sutures and the muscle's insertion. The
operative tissue reaction. Others say that an unsight- needles are then passed through the tendon at the
ly lump is produced. I use this procedure when it is insertion. The sutures are tied, bringing the point of
necessary to preserve anterior segment circulation for suture placement up to the insertion creating a loop of
any reason. Theoretically, a plication should be as redundant muscle-tendon and shortening of the mus-
effective as a similar sized resection. I have per- cle. The redundant loop of muscle-tendon is sutured
formed too few of the procedures to personally con- to the muscle to reduce the bulk (Figure 11).
firm this, but I believe it is true. This technique can
be used on any of the rectus muscles.

210
Resection of a rectus muscle

A B

C D

Figure 11
A A double arm suture is placed at each muscle border at C The sutures are tied creating a loop.
the point equal to the intended muscle shortening and D The tip of the loop is secured to the top of the muscle.
are secured with a knot.
B The sutures are brought through the insertion of the
muscle.

Displacement of horizontal
rectus muscles with resection
for A and V patterns
As with all vertical displacements of the hori- and the lateral rectus muscles weakened for exodevi-
zontal rectus for treatment of vertically incomitant ations.
strabismus, the lateral rectus muscles are moved A recession-resection procedure is carried out
toward the open end of the pattern, and the medial on the left eye of a patient with a V pattern exotropia.
rectus muscles are moved toward the closed end of The resected left medial rectus is shifted one-half
the pattern. The surgeon needs to remember that muscle width downward, and the recessed left lateral
medial rectus muscles are weakened and the lateral rectus is shifted one-half muscle width upward
rectus muscles strengthened for esodeviations; con- (Figure 12).
versely, the medial rectus muscles are strengthened

211
Chapter 8

Figure 12
A The medial rectus is resected and moved down while the B The resected medial rectus is moved up and the
lateral rectus is recessed and moved up to treat a V XT. recessed lateral rectus is moved down for an A XT.

Vertical effect from horizontal


rectus resection and recession
When performing a resection-recession proce- The muscles are moved upward one-half muscle
dure on a patient who also has a small to moderate width or more if the eye is hypodeviated and down-
vertical deviation, both rectus muscles may be shifted ward one-half muscle width or more if the eye is
vertically in the same direction. This approach can hyperdeviated (Figure 13).
treat the vertical deviation without altering the effect
of the procedure for the esodeviation or exodeviation.

Figure 13
A The eyes are ET 40D with a right hypotropia of 15D.
continued.

212
Resection of a rectus muscle

Figure 13, contd


B The medial rectus is recessed 5 mm (or approximately D The eyes are XT 40D with a right hypertropia of 15D.
10.5 mm from the limbus) and the lateral rectus is resect- E The lateral rectus is recessed 7 mm and the medial rec-
ed 8.5 mm. Both muscles are shifted one-half muscle tus is resected 8 mm. Both muscles are shifted down-
width upward. ward.
C The eyes should be better aligned vertically and horizon- F The eyes should be better aligned vertically and horizon-
tally after surgery. tally after surgery.

213
9
Surgery of the obliques

Oblique muscle surgery


The superior oblique is the muscle most fre- as occurs in some cases of congenital superior
quently affected in acquired extraocular muscle palsy oblique palsy. This phenomenon was initially noted
at least as seen by the strabismologist. In the treat- in cases with severe craniofacial abnormality. Later it
ment of superior oblique palsy most of the surgical was found in patients who were thought simply to
activity involves the other oblique muscle, the inferi- have congenital superior oblique palsy. Patients with
or oblique. For most indications, myectomy, reces- congenital superior oblique palsy who are subse-
sion, or anterior transposition of the inferior oblique quently found to have absence of the superior oblique
are the most commonly performed oblique muscle tendon are likely to have one or more of the follow-
surgical procedure. Inferior oblique weakening is ing features: horizontal strabismus, head tilt, ambly-
commonly performed, usually bilaterally, to treat opia, facial asymmetry (with the fuller face on the
what is called primary overaction of the inferior side of the absent tendon), and marked underaction of
obliques and also for inferior oblique overaction after the superior oblique.
bimedial rectus recession. Both demonstrate strabis- In addition to these clinical findings that sug-
mus sursoadductorius (elevation in adduction) and gest a tendon anomaly, traction testing of the superi-
produce a V pattern. or oblique tendon in the operating room at the time of
There are two important reasons why inferior surgery must be done to further identify tendon laxi-
oblique weakening is the surgery of choice in superi- ty. This test should be done in all patients with supe-
or oblique palsy. The first and most important reason rior oblique palsy undergoing surgery. The superior
is to avoid attempts at strengthening the superior oblique traction test provides reliable information
oblique. Because the reflected tendon of the superior about the state of the superior oblique tendon; infor-
oblique has a limited potential amount of slack or mation that is essential for the design of the appropri-
redundancy, especially in acquired superior oblique ate surgical procedure. In cases with laxity of the ten-
palsy, tuck or resection of this tendon can cause an don, which is the hallmark of congenital superior
iatrogenic Brown's syndrome (restricted elevation in oblique palsy, the superior oblique traction test will
adduction). The second reason is that weakening of be positive for a loose tendon. This test is deemed
the inferior oblique muscle is simple, effective and positive if the globe is retroplaced easily in the orbit
predictable. and the normal taut superior oblique tendon with the
Surgical treatment of superior oblique palsy is globe popping over is not felt. Instead, a less dis-
usually indicated in unilateral cases because of tinct or soft tendon band or perhaps no tendon band
asthenopia or constant or intermittent diplopia with or at all is felt. Strengthening procedures on the superi-
without head tilt. Bilateral superior oblique palsy is or oblique should be done only in cases where laxity
treated surgically because of nonfusable torsional of the tendon can be confirmed by this test or in
diplopia and/or severe chin depression to deal with selected cases with severe torsion. A positive (loose)
the V pattern. traction test followed by exploration confirming that
The superior oblique is the extraocular muscle the tendon is anomalous confirms that the superior
most likely to be anomalous. These anomalies range oblique palsy is of congenital origin, in my opinion.
from laxity or redundance to a misdirected insertion However, the question remains in the mind of some:
or even absence of the reflected tendon and trochlea, Could prolonged paresis lead to secondary laxity or

215
Chapter 9

elongation of the tendon? For congenital superior A half century ago, the superior oblique was
oblique palsy with a lax or misdirected tendon, I do a described as nolo tangere or do not touch. This
strengthening or shortening procedure of the superior advice is no longer valid, but I would replace this
oblique tendon. If the tendon is absent, the antago- advice with the recommendation for superior oblique
nist, or yoke muscle, is weakened and, in some cases, surgery, handle with care. In contrast, the inferior
the ipsilateral superior rectus is weakened. oblique is weakened frequently and in most cases
A newer oblique muscle procedure is the ante- results are good. While the superior and inferior are
rior transposition of the inferior oblique. This proce- both oblique muscles, the similarity ends there.
dure is performed in cases of overaction of the inferi-
or obliques with V pattern and dissociated vertical Weakening the inferior oblique
deviation. The new insertion of the inferior oblique is
placed just anterior to the temporal corner of the infe- Inferior oblique myectomy
rior rectus insertion. In addition to weakening inferi- When performed carefully, an inferior oblique
or oblique action, it seems to have a tethering effect myectomy can be completed with little or no bleeding
on the upward movement of the globe and therefore and fat should never be encountered. Because the
lessens the amplitude of vertical deviation of the inferior temporal vortex vein may be encountered, it
DVD. must be dealt with carefully to avoid rupture. If the
Stager has devised a procedure placing the new vein is severed, copious bleeding will occur. This
insertion of the inferior oblique nasal to the inferior would be controlled with tamponade and/or cautery.
rectus. This changes the inferior oblique from an Given the more or less blind sweep of the hook it is a
extorter to an intorter and is therefore potentially use- pleasant and certainly welcome occurrence that vor-
ful in cases with large extorsion. tex vein rupture in this area occurs so infrequently. In
Other surgical procedures of the obliques nearly 40 years, I have not seen this.
include weakening procedures of the superior oblique Persistent inferior oblique overaction can result
usually performed by means of tenectomy, tenotomy, if a slip of muscle is not cut or if the proximal cut end
or disinsertion. Marginal tenotomy of the superior of the inferior oblique attaches to the sclera resulting
oblique has been described, but it is not a logical in scarring and fibrosis. This is avoided by tucking
choice because it is either not effective or it becomes the proximal end of the inferior oblique behind poste-
a complete tenotomy because of the cable-like make- rior Tenon's capsule.
up of the tendon. A few surgeons prefer to weaken The incision for exposure of the inferior
the superior oblique by recession. No clear-cut dif- oblique muscle is approximately 8 mm long. It is
ference in results of these weakening procedures has located 8 mm from the limbus and is concentric with
been provided. The complex anatomy of the superior it . It is also anterior to the inferior fat pad (Figure 1).
oblique tendon as it relates to the superior rectus and The eye is stabilized in elevation and adduction with
superior orbital fascia contributes to the differing a locking forceps. One or two fine-toothed forceps
results from the various weakening procedures. are used initially to elevate the conjunctiva, Tenon's
Transfer of the superior oblique tendon, with- capsule, and intermuscular membrane, and a snip
out fracture of the trochlea, is accomplished in some incision is made between the forceps, exposing bare
cases of third nerve palsy. Fracture of the trochlea to sclera (Figure 2). Blunt-tipped Wescott scissors are
achieve removal of the tendon is, in my opinion, not inserted into the incision against bare sclera and the
practical and should not be attempted. Shift of the scissors tips are spread to separate with blunt dissec-
anterior insertion fibers (or the entire insertion) is per- tion the filamentous attachments between the sclera
formed to enhance intorsion in selected cases of supe- and posterior Tenon's capsule.
rior oblique palsy, especially in bilaterally involved To expose the anterior border of the inferior
individuals with torsional diplopia. oblique muscle, the surgeon first places a large mus-
A technique for treating Brown syndrome cle hook behind the insertion of the lateral and the
employs a band of silicone used as an expander to inferior rectus muscles. Then a third muscle hook is
lengthen the superior oblique tendon nasal to the used to elevate the posterior border of the conjuncti-
superior rectus. A variety of procedures for weaken- va - Tenon's capsule incision. Deep in the incision at
ing the superior oblique in cases of Brown syndrome the junction of the sclera and posterior Tenon's cap-
are done, ranging from disinsertion of the posterior sule, the anterior border of the inferior oblique will be
seven-eighths of the insertion to tenectomy near the seen.
trochlea. However, any technique can fail in some A small hook is placed flat against sclera,
cases and succeed in others. This tells us that no sur- indenting it slightly with the tip toward the inferior
gical procedure or strabismus surgeon (at least that I rectus (or toward the lateral rectus). The hook is gen-
have heard of) has the surgical answer for all cases of tly slid beneath the inferior oblique muscle until the
Brown syndrome. orbital wall is felt. The tip of the hook is then rotated

216
Surgery of the obliques

A B

Figure 1
A The site of the incision for exposing the inferior oblique. B A snip incision going through conjunctiva, anterior
Tenons capsule, and intermuscular membrane exposes
bare sclera.

A B

C D

Figure 2
A Blunt dissection frees intermuscular membrane from C A small hook slides posteriorly along sclera and is rotat-
sclera. ed when it is behind the posterior border of the inferior
B Large muscle hooks are placed behind the insertion of oblique.
the lateral and inferior rectus and a small hook lifts inter- D The small hook brings the inferior oblique forward.
muscular membrane exposing the anterior border of the
inferior oblique.

217
Chapter 9

until it points at the junction of the lateral and inferi- is included and avoiding making a hole in the inter-
or orbital rim. As the inferior oblique is engaged, the muscular membrane (posterior Tenon's capsule)
hook retracting conjunctiva is pulled back to expose which would result in prolapse of orbital fat (Figure
the tip of the small hook that shows through Tenon's 3). Fortunately, the inferior temporal vortex vein is
fascia and is beyond the posterior muscle border. very infrequently ruptured. However, excess blind
When the inferior oblique muscle is engaged on the manipulation in this area should be avoided to lessen
teaser hook, the surgeon must take great care to bring the chances of this annoying complication.
the muscle forward, making sure that only the muscle

A B

C D

Figure 3
A The tip of the small hook is seen behind the posterior D The hooks, or larger hooks that have replaced the small
border of the inferior oblique. hooks, are rotated to inspect the inferior oblique poste-
B A knife or scissor exposes the tip of the hook. rior edge of the inferior oblique with the under surface of
C A second hook is introduced. posterior Tenons capsule behind.
E If some inferior oblique muscle is seen behind the hooks,
it is carefully included by placing a second pair of hooks.

218
Surgery of the obliques

A scissors or a scalpel blade is used to cut indicates that the entire inferior oblique has been
down on the tip of the small hook, exposing it behind engaged. At this point a vortex vein will be seen leav-
the inferior oblique. A second hook is placed and the ing sclera and passing through intermusuclar mem-
fascial layers associated with the muscle are dissect- brane. If the inferior oblique has been hooked incom-
ed from the muscle, exposing 5 to 8 mm of the infe- pletely, a red stripe will be seen horizontally below
rior oblique. The muscle hooks behind the insertions the hooks. This strip of muscle should be picked up
of the lateral and inferior rectus muscles may be gently with two small hooks, repeating the procedure
removed as soon as the second hook is placed under until the entire inferior oblique muscle has been
the inferior oblique. The small hooks under the infe- engaged. Two hemostats placed 5 to 8 mm apart are
rior oblique are replaced by two larger hooks, which used to clamp the inferior oblique muscle belly. With
are rotated away from the scleral surface and the scissors or a scalpel blade, a 5 to 8 mm segment of the
undersurface of the inferior oblique. At this time it inferior oblique muscle belly lying between the
can be determined whether the entire inferior oblique hemostats is excised. Cautery is then applied heavily
has been engaged. A clearly defined border of the to each cut end for hemostasis (Figure 4).
inferior oblique with white Tenon's capsule below

A B

C D E

Figure 4
A Hemostats are placed with a 5-8 mm segment of muscle D The small hole in posterior Tenons capsule can be
between. closed with an 8-0 absorbable suture.
B After the segment of muscle has been cut out with scis- E Conjunctiva is closed with an 8-0 absorbable suture.
sors or a scalpel, the cut ends are cauterized.
C The inferior oblique is allowed to retract or the inferior
oblique may be nudged into the hole in Tenons.

219
Chapter 9

After the hemostats are removed, the inferior Alternative weakening procedures of
oblique muscle is allowed to retract and the conjunc- the inferior oblique: recession and
tival incision is closed with either interrupted or run- disinsertion
ning sutures, or this incision may be left unsutured,
depending on the surgeon's preference. If the proxi- Two other techniques for weakening an over-
mal cut end of the inferior oblique fails to retract into acting inferior oblique are recession and disinsertion.
the space outside posterior Tenon's capsule, it can be Recession of the inferior oblique can be graded and is
nudged into the space with the tip of a muscle hook especially useful in cases where a minimal amount of
or forceps. As the inferior oblique retracts into the weakening is required. Because the new inferior
defect in Tenon's capsule and approaches the lateral oblique insertion is placed at a specific point on the
border of the inferior rectus, a small slit in posterior globe after recession, finding the inferior oblique at a
Tenon's capsule can be seen. This slit can be closed subsequent operation is easier to accomplish than
with one or two 8-0 Vicryl sutures. after myectomy or disinsertion.
The incision, localization, and exposure for
A common complication of inferior recession or disinsertion of the inferior oblique mus-
oblique weakening and how to avoid cle are the same as described previously for a myec-
it tomy. Recession of the inferior oblique muscle is
begun by placing either two single-arm sutures or a
As is the case with any of the extraocular mus- single double-arm suture through the inferior oblique
cles, the function of the inferior oblique depends on muscle near the lower border of the lateral rectus.
its having some tissue connecting the origin and (Figure 5). The suture is therefore placed approxi-
insertion. Contraction of the muscle causes these two mately a millimeter from the muscle's broad inser-
points to be brought closer together. The muscle's tion. To ensure inclusion of all of the muscle fibers at
effect is manifested through movement of the globe this point, careful inspection of the posterior border of
toward the fixed point or origin of the muscle. the inferior oblique insertion should be carried out.
If in the process of performing a myectomy or The surgeon must detach the entire width of the infe-
any weakening procedure of the inferior oblique mus- rior oblique muscle, freeing the muscle completely
cle the clamps or recession sutures exclude a portion from the sclera for the recession to be effective.
of the muscle, a band of uninterrupted muscle tissue The inferior oblique is reattached to the sclera
with associated intermuscular membrane remains at a point that depends on the amount of recession
connecting origin and insertion. A portion of the infe- intended. Fink described an instrument for locating
rior oblique coursing uninterrupted between origin the point of reinsertion (see page 12), but recession is
and insertion acts somewhat like a tendon. Inferior now usually accomplished by reattaching the inferior
oblique weakening would then be less than expected. oblique in relation to existing landmarks. For exam-
This complication which causes undercorrec- ple, Parks reattached the anterior corner of the inferi-
tions can be avoided. Careful inspection of the pos- or oblique 2 mm lateral and 3 mm posterior to the lat-
terior aspect of the inferior oblique muscle reveals eral border of the inferior rectus insertion. The poste-
any remaining bands. These bands are engaged on rior scleral reattachment is placed according to the
muscle hooks and a myectomy is repeated on this width of the inferior oblique muscle. Other tech-
smaller segment of the inferior oblique muscle. For niques reinsert the inferior oblique slightly more pos-
an inferior oblique myectomy to be effective, a seg- teriorly. Regardless of the intended amount of reces-
ment of inferior oblique that includes its entire width sion, when the line of pull of the inferior oblique is
must be removed. A partial myotomy of the inferior maintained the new effective insertion is at the later-
oblique in my experience is ineffective. When disin- al border of the inferior rectus. Changing the line of
sertion of the inferior oblique is chosen for weaken- pull of the inferior oblique, as in anterior transposi-
ing this muscle, care must be taken to sever the entire tion, both weakens the pull of the inferior oblique
insertion. Some surgeons perform marginal myoto- and tethers or mechanically limits elevation.
my of the inferior oblique and claim good results. I do Another technique for weakening the inferior
not recommend this procedure. oblique is disinsertion. In this procedure, after iden-
tifying and hooking the inferior oblique in the usual
manner, the insertion of the inferior oblique is
exposed while the lateral rectus is elevated on two
muscle hooks. The inferior oblique is detached from
the sclera. The muscle is allowed to retract and the
incision is closed (Figure 6).

220
Surgery of the obliques

A B

Figure 5
A For recession, after exposing the inferior oblique, the lat- B The muscle is cut from the globe.
eral rectus is lifted and one or two sutures are placed in C The muscle is reattached along the line of pull approxi-
the inferior oblique one or two millimeters from the inser- mately 6 to 8 mm from the insertion.
tion.

A B

Figure 6
A For disinsertion, after exposing the inferior oblique in the B The inferior oblique is cut at its insertion and is allowed
usual way, the lateral rectus is elevated on two hooks to retract.
exposing the inferior oblique insertion.

221
Chapter 9

Extirpation of the inferior oblique


Gonzales described an alternative technique
for weakening the inferior oblique that involved sev-
ering the nerve, a heavy structure that enters the infe-
rior oblique at its posterior border as it crosses the
inferior rectus. This nerve was cut and cauterized, but
it grew back with time and inferior oblique function A
returned. Realizing this, he took the next step and
removed as much of the distal inferior oblique as pos-
sible resulting in what was called denervation and
extirpation. The procedure was designed to treat and
or prevent persistent overaction of the inferior
oblique.
It did not gain widespread popularity. A possi-
ble reason is that other surgeons may not have had the
same experience with persistent overaction of the
inferior oblique after weakening. Parks redescribed
extirpation of the inferior oblique in a manner nearly
identical to that of Gonzales. It has been suggested
that what some surgeons diagnose as persistent over-
action of the inferior oblique actually may be unrec-
ognized dissociated vertical deviation. B
Extirpation of the inferior oblique which is
really removal of the distal muscle and nerve seems
unnecessarily ambitious when a simple procedure
produces excellent results. Stager has added myecto-
my of the nasal portion of the inferior oblique to ante-
rior transposition to weaken the inferior oblique while
retaining depressor effect to treat dissociated vertical
deviation.

Suture traction for exposure


For any procedure on the inferior oblique
including extirpation, measured recession, or anterior
Figure 7
transposition, exposure can be obtained using a heavy A After incision through the layers overlying sclera, a 4-0
traction 4-0 or 5-0 silk suture under the lateral rectus black silk suture is placed behind the lateral rectus.
insertion. It is placed behind the insertion of the lat- B Two hooks lift the inferior oblique, a spatula indents scle-
eral rectus with the heavy needle brought through the ra, and a small hook slides behind the inferior oblique.
incision, behind insertion, and then through Tenon's
capsule and the conjunctiva above (Figure 7).
To gain better exposure of the inferior oblique Anterior transposition of the inferior
at the insertion, two large hooks spread the insertion, oblique
traction is placed on the black silk traction suture, and
a spatula is used to indent sclera enabling placement Anterior transposition of the inferior oblique
of a small hook which will engage the inferior was described by Elliott and Nankin. The effect of
oblique. The inferior oblique muscle is dissected and this procedure is, first, to weaken the inferior oblique
pulled out of the incision (Figure 8). The insertion of eliminating so-called overaction that causes strabis-
the inferior oblique muscle is exposed and a hemostat mus surso adductorus (elevation in adduction) and
is placed across the muscle. At this point the inferior V pattern; and, second, to restrict the supraduction
oblique is detached flush with sclera. If recession or from dissociated vertical deviation. This surgery
anterior transposition is performed, a suture (6-0 shifts the new insertion of the inferior oblique adja-
Vicryl) is placed and the procedure is carried out as cent and just anterior to the ipsilateral inferior rectus
described. If extirpation and denervation is the insertion. The most likely reason for the reduction of
choice, the inferior oblique and its nerve are cut. The the dissociated vertical deviation after anterior trans-
nerve retracts and the muscle is removed. The defect position of the inferior oblique is the mechanical or
in Tenon's capsule may be closed with one or two fine tethering effect of the newly placed inferior oblique
absorbable sutures. insertion mediated by the stout nerve to the inferior

222
Surgery of the obliques

A B

C D

Figure 8
A The insertion is exposed. C The muscle is detached and from here disinsertion,
B The muscle is clamped near the insertion. recession, myectomy or anterior transposition can be done.
D conjunctiva is closed.

oblique. It is unlikely, in my opinion, that the inferi- just temporal and anterior to the inferior rectus inser-
or oblique is actually converted to a depressor. tion. The tip of the inferior oblique is sutured to scle-
The indication for anterior transposition of the ra 1 or 2 mm anterior to the lateral border of the infe-
inferior oblique is overaction of the inferior obliques rior rectus insertion, immediately adjacent to the infe-
causing excess elevation in adduction and a V pat- rior rectus.* The new inferior oblique insertion
tern plus dissociated vertical deviation. This proce- should be just anterior but parallel to the inferior rec-
dure should not ordinarily be performed on just one tus insertion. The conjunctiva may be closed with
eye because of the possibility of producing a large one or two 8-0 Vicryl sutures. A mound of inferior
secondary deviation. I have done it in one patient, but oblique muscle, obvious just behind the limbus
soon after this procedure did it in the other eye. immediately after surgery, subsides in a few weeks
To perform anterior transposition, the inferior and does not present a problem. But, fullness of the
oblique muscle is isolated and a 6-0 Vicryl suture is lower lid persists in some cases.
placed at the distal end of the inferior oblique (Figure
9). A large hook is then placed to expose the sclera

*Surgeons differ in their choice for positioning the new insertion of the inferior oblique, placing it even with the inferior rectus or a mil-
limeter behind.

223
Chapter 9

A B

D
C

Figure 9
A A suture is placed at the distal inferior oblique and the D The level of attachment of the inferior oblique can vary
muscle is detached. from a few millimeters ahead of to a few millimeters
B A hook is placed behind the inferior rectus insertion... behind the level of the inferior rectus insertion.
C ...and the inferior oblique is secured to sclera. E The conjunctiva is closed.

224
Surgery of the obliques

Strengthening the inferior


oblique
Inferior oblique tuck: resection and
advancement of the inferior oblique
Strengthening procedures on the inferior than 10 mm (5 mm up and 5 mm down) of the mus-
oblique are the least effective types of surgery for the cle should be included.
vertically acting muscles and are rarely indicated. For resection and advancement of the inferior
However, the procedure is performed occasionally in oblique, two single-arm sutures are placed at the bor-
the rare case of inferior oblique palsy that is not ade- ders of the inferior oblique muscle just below the
quately treated by weakening the yoke superior rec- inferior border of the lateral rectus - approximately 5
tus. Two techniques for strengthening the inferior mm from the insertion (Figure 11). The muscle is
oblique, tuck and resection with advancement, have clamped with a hemostat and is severed just distal to
been described. the sutures. The muscle stump is cut free from the
To tuck the inferior oblique, the muscle is first globe at the insertion and is discarded. The inferior
localized and engaged in the inferior temporal quad- oblique is reattached to the sclera at the upper border
rant exactly as it is done before performing a myecto- of the lateral rectus. The anterior suture is placed 12
my (Figure 10). A tuck is then made in the muscle mm posterior to the lateral rectus insertion and the
using a Fink tucker or a freehand technique can be posterior suture is placed slightly more posterior.
used. Nonabsorbable suture such as 5-0 Mersilene is This produces approximately 10 mm or slightly more
used to secure the tuck. When a tuck is made, no less resection effect.

A B

Figure 10
A A tucker is placed. B The loop is 5 mm on each side of the tucker (10 mm
total). It is secured with 5-0 non-absorbable suture.

A B

Figure 11
A Sutures are placed at the borders of the inferior oblique. B The inferior oblique is detached and reattached at the
upper border of the lateral rectus.

225
Chapter 9

Weakening procedures of the superior


oblique/tenotomy
The superior oblique is effectively weakened
by a tenectomy, tenotomy, or recession. For best
results, these procedures should be performed with A
minimal disruption of the orbital fascial layers sur-
rounding the superior oblique tendon.
The incision for exposure of the superior
oblique tendon medial to the superior rectus is begun
at the medial aspect of the superior rectus muscle
insertion and extends through the conjunctiva, anteri-
or Tenon's capsule, and intermuscular membrane 8
mm from and concentric with the limbus (Figure 12).
When bare sclera is exposed, moderate-sized muscle
hooks are placed behind the insertion of the superior
rectus and the medial rectus and a third muscle hook
is placed beneath the posterior free edge of the inci-
sion to retract the intermuscular membrane, anterior
Tenon's capsule, and conjunctiva. These three muscle
hooks are held under slight tension to produce an
incision the shape of an equilateral triangle. The sur- B
geon then observes the undersurface of posterior
Tenon's capsule. A whitish band will be seen; this is
the superior oblique tendon encased in orbital fascia.
The width of the superior oblique tendon here is
approximately 3 mm. A small right angle hook is
placed into the incision to engage the superior oblique
tendon with a minimum of associated fascia. The tip
of the hook is dissected free with scissors or a scalpel
blade so that it projects cleanly behind the posterior
aspect of the superior oblique tendon (Figure 13).
A second hook is placed beneath the superior
oblique tendon. The anterior aspect of the associated
fascia is dissected from the tendon along the long axis
of the tendon. A third hook is placed beneath the ten-
don but remains superficial to the associated fascia.
Another teaser hook engages only the tendon and the
two hooks beneath the combined tendon and fascia
are removed. Scissors are used to cut the superior C
oblique tendon.
Before cutting the superior oblique tendon, the
surgeon must decide on the degree of weakening
intended. A tenotomy adjacent to the medial border
of the superior rectus muscle, closer to the insertion,
results in less weakening. A tenotomy carried out
closer to the trochlea produces more weakening.
Grading of a superior oblique weakening procedure is
achieved in this manner rather than by varying the
size of a tenectomy. The actual degree of weakening
in a superior oblique tenectomy is governed by the Figure 12
A Site of the incision for exposing the superior oblique
proximity of the nasal end of the tenectomy to the nasal to the superior rectus in the left eye.
trochlea rather than by the size of the segment of ten- B Hooks are placed behind the superior and medial recti
don removed. Therefore, a tenotomy is sufficient. and elevating intermuscular membrane. The white band
With the tenotomy completed, the tendon retracts. of the superior oblique tendon is imbedded in the fascia
seen through intermuscular membrane.
The incision is closed with one or more absorbable C A small hook picks up the superior oblique tendon. The
sutures. tip of the teaser hook is covered by orbital fascia.

226
Surgery of the obliques

A B

More
C D weakening

Less
weakening

F
E

Figure 13
A The tip of the hook behind the tendon and fascia is D The location of the cut influences the amount of weaken-
exposed with a scissors or scalpel blade ing.
B The fascia is carefully separated from the tendon fibers E The tendon retracts.
and a hook is placed behind the isolated tendon. F The conjunctiva is closed.
C A second hook is placed behind the tendon and the ten-
don is cut.

227
Chapter 9

Superior oblique tenotomy after a


temporal approach
An alternate method for performing tenotomy rior muscle capsule beneath the superior rectus mus-
of the superior oblique uses exposure of the superior cle. Because the superior oblique tendon is fanned
oblique tendon at its insertion. The superior oblique out at its insertion some fibers may be missed if the
tendon insertion is usually found only after careful only attempt to engage the tendon is made at the
study of the scleral surface reveals the delicate tendon insertion. Careful observation and dissection beneath
fibers as they blend into sclera. A fine hook rubbed the superior rectus will allow complete inclusion of
over the scleral surface can aid in identifying these the more compact superior oblique tendon beneath
fibers. They are located approximately 6 to 12 mm the superior rectus muscle.
posterior to the lateral corner of the superior rectus The superior oblique tendon can be brought
insertion, almost parallel to the lateral border of the temporally while the superior rectus muscle is pulled
superior rectus, approximately at the lateral margin of nasally so that the doubled-over tendon is exposed up
the muscle. to 20 or more mm from the insertion. The tenotomy
For minimal weakening procedures, especially may now be carried out. The principle of obtaining
to reduce a small A pattern bilateral disinsertion of more effect from tenotomies closer to the trochlea and
the posterior seven-eighths of the superior oblique less effect from tenotomies farther from the trochlea
insertion can be effective. Preito-Diaz recommends remains valid using the superior oblique exposure
removing a triangle of superior oblique insertion with from the temporal approach. Because up to 20+ mm
the apex pointing toward the trochlea. This procedure of the superior oblique tendon can be exposed tempo-
may also be used in selected cases of Brown's syn- ral to the superior rectus, measuring or estimating the
drome, provided this minimal procedure allows free, distance from the insertion to the point of tenectomy
unrestricted passive elevation in adduction confirmed actually gives a more reliable measure of the tenoto-
at surgery. my. Because the superior oblique tendon will not
To carry out surgery on the superior oblique at stretch, the surgeon should be able to perform a repro-
the insertion, the superior rectus tendon is engaged ducible tenotomy using a temporal approach.
from its temporal side with a large muscle hook. A
second muscle hook elevates the superior rectus mus-
cle approximately 5 to 8 mm posterior to its insertion
and a third muscle hook retracts the conjunctiva and
anterior Tenon's capsule and intermuscular membrane
laterally and backward (Figure 14). The insertion of
the superior oblique tendon is searched for carefully.
At this point a minimum of manipulation should be
carried out. Careful blotting with a cotton-tipped
applicator and slight manipulations of the globe will
reveal the insertional fibers of the superior oblique
tendon fusing with the sclera approximately at right A
angles to the superior oblique insertion.
Anterior fibers of the superior oblique insertion
are engaged with a small muscle hook. Hooking at
least part of the superior oblique tendon allows the
tendon to be pulled temporally while the superior rec-
tus is pulled nasally, exposing the thin cord-like prox-
imal portion of the superior oblique tendon that can
be engaged with a small hook. The first hook is
removed after a second hook has been placed behind
the proximal portion of the superior oblique tendon.
A larger muscle hook replaces the two small Figure 14
hooks and is then used to engage the superior oblique A The three hooks create a triangular incision exposing the
tendon and bring it temporally. At this point scissors site of the superior oblique insertion.
can be used to dissect the superior oblique tendon free continued.
from the intermuscular membrane fibers and the infe-

228
Surgery of the obliques

B C

D E

F G

Figure 14, contd


B The tendon (or at least the anterior part of it) is hooked. F The tendon is cut.
C A second hook engages the round tendon proximally. G The farther the tenectomy from the insertion (closer to
D Another hook is placed under the tendon. the trochlea), the greater the weakening.
E After a larger hook replaces the two smaller ones, the
tendon is pulled temporally while the superior rectus is
pulled nasally.

229
Chapter 9

7/8 tenotomy/disinsertion
After exposing the insertion of the superior from its insertion, and a surgeon's knot is tied. This is
oblique tendon, scissors are used to disinsert the ten- to ensure that the suture is placed in solid tendon. The
don or to carry out a 7/8 posterior tenotomy of the tendon is then transected between the suture and the
superior oblique tendon with excision of a triangle tendon's insertion. The tendon is allowed to retract
shape portion of the tendon at the insertion (Figure beneath the superior rectus for a distance of 8 to 20
15). mm, according to the intended amount of recession,
and the suture is tied in a hang loose fashion at the
Recession of the superior oblique tendon insertion (Figure 16).
For a more controlled, weakening of the supe-
rior oblique tendon, recession in the place of tenoto-
my can be done. Split tendon lengthening of the
superior oblique tendon nasal to the superior rectus
has been described and is performed by some sur-
geons in selected cases. It has also been performed
and abandoned by other surgeons. While this tech-
nique is theoretically possible, it is difficult to per- A
form. I see no reason to use it in place of other avail-
able superior oblique weakening procedures and for
that reason it will not be illustrated.
In preparation for recession, the superior
oblique tendon is located and engaged at its insertion
temporal to the lateral border of the superior rectus.
A double-arm 5-0 Mersilene or 6-0 Vicryl suture is
placed through the superior oblique tendon 4 mm

B
A

Figure 16
A The superior oblique tendon is exposed at the insertion
temporal to the superior rectus.
B A double arm suture is placed 4 mm from the insertion
Figure 15
and the tendon is cut free of sclera at the insertion.
Disinsertion - 7/8 tenotomy C The needles are brought through the insertion. After the
A Posterior 7/8 tenotomy tendon is allowed to retract a graded amount according
B Disinsertion to the amount of weakening needed, the suture is tied.

230
Surgery of the obliques

Sagittalization of the superior


oblique
Anterior shift of the superior oblique tendon The superior oblique tendon is exposed at its
insertion has been advocated for treatment of the tor- insertion and a small hook engages all or part of the
sional diplopia resulting from some superior oblique insertion. The superior rectus is retracted medially, a
palsy. Harada and Ito are given priority for describ- 6-0 Vicryl or 6-0 non-absorbable suture is tied to the
ing this technique. The intorting power of the superi- superior oblique tendon close to its insertion, the
or oblique is increased by: anterior and temporal shift superior oblique tendon is split, and the anterior half
of the anterior part of the insertion; anterior shift of of the superior oblique tendon is detached from the
the entire insertion; or anterior shift of the tendon globe (Figure 17). The anterior half of the superior
without disinsertion. This moves the superior oblique oblique tendon is sutured to the sclera 5 to 8 mm ante-
insertion so that it has more effect temporal to the Y riorly near the temporal aspect of the superior rectus
axis of the eye, thereby increasing the superior or it may be placed near the superior border of the lat-
obliques effect as an intorter. eral rectus for more effect. The anterior superior
The superior oblique tendon normally inserts oblique fibers are probably more effective intorters
in the posterotemporal quadrant of the top of the and the posterior superior oblique fibers are more
globe. It functions as a depressor, abductor, and effective depressors. A specific and selective weak-
intorter. By shifting the anterior half of the tendon 5 ening of depression effect can be obtained, at least
to 8 mm anteriorly and a few millimeters temporally, theoretically, by performing a disinsertion of the pos-
the intorting action of the superior oblique is terior fibers of the superior oblique tendon's insertion.
enhanced without affecting appreciably the other Wheeler suggested this procedure in 1935. Metz sug-
superior oblique functions. gested an adjustable anterior shift and advancement
of the superior oblique tendon.

A B

C D

Figure 17
A The superior oblique inserts in the posterior temporal C A double arm suture is placed into the tendon (right eye,
quadrant. surgeons view).
B The anterior (or if you choose the entire) tendon is D The tendon is secured to sclera
engaged on a hook (right eye, surgeons view).

231
Chapter 9

Brown's superior oblique tendon


sheath syndrome
Brown's superior oblique tendon sheath syn-
drome or simply Brown syndrome (eliminating the A
reference to etiology) is characterized by inability on
a mechanical basis of the eye to elevate normally in
the adducted position. It is usually unilateral and spo-
radic but it may occur in one or both eyes and has
been seen in blood relatives. This condition is also
associated with widening of the palpebral fissure on
attempted elevation and occasionally there is down-
shoot of the eye in adduction. The diagnosis is con-
firmed only after demonstration of limited forced ele-
vation, especially in adduction. This is done at the
time of surgery since most patients treated surgically
for Brown are younger. Brown syndrome may be
congenital or acquired and results from a variety of
causes, but the net result is an inability of the superi-
or oblique tendon to pass freely in its normal range of
motion (16 mm, or 8 mm toward upgaze and 8 mm in
downgaze) through the trochlea - the upgaze motion B
being necessary to allow the eye to elevate in adduc-
tion. Unless the superior oblique tendon can pass
freely through the trochlea, the distance between the
trochlea and the insertion cannot increase and the eye
cannot be elevated in adduction.
The surgical treatment of Brown syndrome can
be one of the more frustrating of the extraocular mus-
cle procedures because surgical attempts designed to
reduce the mechanical restriction are often nullified
by postoperative adhesion formation. On the other
hand, when successful, surgery can result in underac-
tion of the muscle. In rare instances the adhesions
that limit elevation in adduction may be associated
with structures other than the superior oblique.
Brown syndrome has been cured by releasing restric-
tions associated with the inferior oblique and also by
lysing a fibrous band along the inferior border of the C
lateral rectus.
As a minimal superior oblique weakening pro-
cedure to treat Brown syndrome, posterior 7/8 superi-
or oblique fiber disinsertion may be performed with
or without removal of a piece of the tendon. I have
treated intermittent Brown syndrome successfully by
excising a clear fluid cyst of the reflected tendon of
the superior oblique near the trochlea, removal of a
fibrous mass at the insertion, tenectomy, and with a
spacer.
Since Brown syndrome represents a wide array
of pathology, no single superior oblique weakening
procedure could be expected to cure or significantly
Figure 18
improve all cases. Actually, some Brown cases could A Intratrochlear adhesions.
not and indeed should not be treated with superior B Tendon shortness - restriction
oblique weakening. An array of potential causes of C Restriction at entry of tendon to trochlea.
Brown are shown (Figure 18). Each of these causes From Helveston EM. Brown syndrome: anatomic considera-
of Brown has a unique personality and requires tions and pathophysiology. American Orthoptic Journal,
unique treatment. 1993, 43:31-35. Reprinted by permission of the University
of Wisconsin Press.

232
Surgery of the obliques

Directed treatment of Brown


syndrome
Brown syndrome should be treated with the
specific technique that would be most likely to effec-
D tively address the unique pathology and after treat-
ment to enable free elevation in adduction. The
important thing for the surgeon to realize is that sim-
ply cutting or lengthening the superior oblique tendon
is not always the answer to Brown syndrome.
Intratrochlear adhesions will impede free pas-
sage of the superior oblique tendon and therefore
restrict elevation in adduction. This is a diagnosis of
exclusion. Weakening of the superior oblique tendon
would be the most appropriate treatment. Superior
oblique palsy is a likely result of freeing elevation in
adduction in a case like this.
Tendon anomaly. This can be a short tendon,
fibrosis of the tendon, or another discrete tendon
abnormality. This is treated by surgery aimed at
undoing the abnormality. This could range from
cutting a fibrous band to cutting the tendon.
Restriction to the tendon entry to the trochlea
E will impede free movement of the tendon through the
trochlea. This is another diagnosis of exclusion. The
eye will not go up in adduction even with forced duc-
tions, but the tendon is normal. Lengthening of the
tendon is the treatment. This is another case where
superior oblique underaction will result after eleva-
tion in adduction is restored.
Inflammation of the trochlea. This is best
treated by the injection of soluble steroid into the
region of the trochlea.
Cyst of the tendon can be treated effectively
with excision of the cyst.
Trochlear trauma (canine tooth syndrome) is
characterized by mechanical limitation of elevation in
adduction and limitation of depression from superior
F oblique palsy. There is no sure treatment. Superior
oblique tenectomy can free elevation in adduction,
leaving superior oblique palsy to be treated.
Because those causes of Brown that require
surgery on the tendon need such varied procedures I
prefer a surgical approach that allows a clear look at
the entire reflected tendon of the superior oblique.
This starts with the cuffed limbal incision and contin-
ues with tagging and detachment of the superior rec-
tus (Figure 19 and see page 176). With this exposure,
the required surgery can be done under direct vision.
Operating with limited exposure and/or simply cut-
ting or otherwise lengthening the superior oblique
Figure 18, contd tendon for all cases of Brown syndrome will produce
D Inflammation, swelling restriction in trochlea. disappointing results in selected cases. To make this
E Cyst at exit of trochlea. surgery even more frustrating, forced duction can be
F Trauma to trochlea - canine tooth. free at surgery only to become restricted again in days
From Helveston EM. Brown syndrome: anatomic considera- or weeks after surgery.
tions and pathophysiology. American Orthoptic Journal,
1993, 43:31-35. Reprinted by permission of the University
of Wisconsin Press.

233
Chapter 9

B
Figure 19
Exposure of the superior oblique from the trochlear cuff to
the insertion after a cuffed limbal incision and tagging and
disinsertion of the superior rectus.

Superior oblique tendon expander


A technique for weakening the superior
oblique tendon using a silicone expander has been
described by Wright. The length of the expander
varies from 4 to 7 mm in 1 mm increments. A 4 mm
expander is used for 1+ and the 7 mm for 4+ superior C
oblique overaction. This technique has been
employed for any type of superior oblique overaction
including Brown syndrome.
The superior oblique tendon is isolated under
direct vision nasal to the superior rectus. The superi-
or oblique tendon is shelled out of the orbital fascia
which is carefully preserved as shown in Figure 13.
Two double-arm 5-0 Mersilene sutures are placed at
mid tendon a few millimeters apart. The superior
D
oblique tendon is cut between these sutures. A No.
240 silicone band is secured with the needles of both
double-arm 5-0 Mersilene sutures. The Mersilene
sutures are tied to secure the silicone expander
between the cut ends of the superior oblique tendon.
This lengthens the tendon. The orbital fascia is
closed over the silicone with 8-0 Vicryl suture.

The chicken suture Figure 20


A The superior oblique tendon is isolated and two double
A procedure for doing a more or less guarded arm merseline sutures are placed a few millimeters
superior oblique lengthening employs a suture apart.
dubbed by Phil Knapp the chicken suture. This is in B The sutures are placed in the ends of a #240 silicone
band.
effect creating controlled tendon expansion with a C The band is secured in place. After this, fascia is closed
suture and muscle contraction in place of the silicone with a 8-0 absorbable sutures.
spacer (Figure 20). D The so-called chicken suture keeps the tendon length-
ening at a specified maximum.

234
Surgery of the obliques

Strengthening the superior


oblique
Superior oblique tuck at the insertion
An effective and safe technique for strengthen- tus (see page 229). If a tendon tucker is used, the
ing the superior oblique is a tuck of the tendon at its hook is replaced with the hook portion of a Bishop or
insertion. This procedure maintains the normal action equivalent tendon tucker.
of the superior oblique muscle and reduces the inci- The knurled knob at the head of the tendon
dence, severity, and persistence of postoperative tucker is screwed down until the slack has been taken
Brown syndrome if appropriate precautions are taken. out of the superior oblique tendon. The total amount
Tuck of the superior oblique tendon should be per- of tendon tucked is twice the amount shown on the
formed only if a loose, lax, or redundant tendon is tucking instrument because the tendon is doubled on
confirmed, first at the superior oblique traction test itself during the tucking procedure. It is impossible to
and then by direct observation. A loose tendon is seen give a number in millimeters for the correct amount
in congenital superior oblique palsy, but usually not of superior oblique tucking in a given case. However,
in acquired superior oblique palsy. After the tuck has it is safe to say that more errors are committed by
been secured, passive ductions should be performed doing too large than too small a tuck. In general, the
testing elevation in adduction. If the superior oblique more vertical deviation to be treated and the more lax
traction test is too tight when comparing it to the fel- the superior oblique tendon the greater the tuck
low eye, the suture securing the tuck should be required. When a sufficient amount of superior
released and the size of the tuck reduced. The tuck oblique tendon has been brought into the tucker to
should be secured only when passive elevation in take out the slack in the tendon, Nonabsorbable suture
adduction is equal or slightly tighter on the tucked (my choice is 5-0 Mersilene) is then used to anchor
side. The size of the tuck depends entirely on the lax- the tuck of the superior oblique tendon. A loop may
ity of the tendon. I once made a 22 mm tuck on a lax be left in the knot securing the tendon at the base of
tendon without producing Brown syndrome in a con- the tuck to facilitate suture release and replacement if
genital superior oblique palsy. On the other hand, I needed. Passive ductions are then performed. The
have produced severe Brown syndrome after a 6 mm tuck is reduced if passive elevation in adduction is
tuck in a case of acquired superior oblique palsy, per- limited. The tuck is made larger if the tendon remains
formed before I became aware of the pitfalls of iatro- lax on superior oblique traction testing.
genic Brown syndrome after tucking a normal (nonre- Each border of the tendon is secured when the
dundant) tendon. tuck is just right, the tucker is removed and the tuck
The incision for exposure of the superior remains intact. A third suture is placed at the apex of
oblique tendon at its insertion is begun at the lateral the tucked tendon, and this tip is attached to the scle-
border of the superior rectus insertion and extends ra in line with the normal pull of the superior oblique
temporally for 8 mm parallel with the limbus. The tendon. The needle should be placed into very super-
initial incision is carried through the conjunctiva, ficial scleral fibers because the sclera can be extreme-
anterior Tenon's capsule, and intermuscular mem- ly thin in this area. The conjunctiva is closed with
brane. A muscle hook is inserted behind the insertion several interrupted sutures.
of the superior rectus muscle and a second hook A tuck of the superior oblique tendon can also
retracts the posterior border of the incision at the lat- be carried out with a free hand technique. With this
eral border of the superior rectus muscle. This technique, the surgeon simply pulls the redundant
maneuver exposes the insertional fibers of the superi- tendon up with a hook and places the sutures through
or oblique tendon. A muscle hook is inserted behind the superior oblique tendon at the level of sclera, pro-
the insertion of the superior oblique tendon and the ducing the intended amount of tuck (Figure 21). This
tendon is brought out from beneath the superior rec- is my preferred technique.

235
Chapter 9

A B

C D

E F

Figure 21
A Incision for exposure of the superior oblique tendon at E The tucker is adjusted pulling the loop of the tendon up
the insertion. until the intended amount of tuck is achieved and a
B Exposing the superior oblique insertion. suture secure the tuck at the borders of the tendon. The
C The superior oblique insertion is engaged on a hook. intended amount of insertion is determined after confir-
D The hook of the tucker engages the tendon. mation that the superior oblique traction test is equal or
slightly tighter on the operated side.
F A second suture secures the tuck and the tip of the loop
is sutured to sclera.

236
Surgery of the obliques

G H

I
Figure 21, contd
G conjunctiva is closed.
H A free hand tuck can be done after pulling up the lax ten-
don. A 5-0 merseline suture joins the arms of the loop
near the base.
I When performing a tuck with either technique, a loop
should be left in the initial knot so that it can be undone
easily in the event that the tuck must be adjusted
because it is too loose or too tight. When the tuck is the
correct amount, the knot is tied and a second suture may
be added.

Superior oblique resection and


advancement
Other techniques for strengthening or shorten- suture. The hemostat holds the distal superior oblique
ing the superior oblique tendon are resection, tendon, stabilizing the insertion. The double-arm
advancement, or resection and advancement. For Vicryl suture attached to the proximal tendon is
superior oblique resection, I prefer to attach the prox- brought through the insertion at the middle, posterior
imal tendon to the middle, posterior, or anterior inser- edge, or anterior edge. The middle is selected if the
tion with placement depending on the amount of pre- torsional and vertical defects are proportional, poste-
operative torsion. One reason for choosing tuck over rior if the vertical defect is greater, and, as occurs
resection for the superior oblique is that it can be dif- more commonly, anterior if extorsion is the main pre-
ficult to place sutures in the thin superior oblique ten- operative problem. The tendon can be advanced
don. (attached farther temporally) or shifted anteriorly if
The superior oblique tendon is exposed at the more torsional effect is needed. Also, any of these
insertion (Figure 22). The tendon is engaged on a procedures can be performed with an adjustable
hook near its insertion and the superior rectus muscle suture. Anteriorly placed sutures are more readily
is retracted medially. A 6-0 Vicryl or 5-0 merseline adjusted than posteriorly placed sutures.
suture is woven through the tendon 6 to 10 mm or As with any procedure to strengthen the supe-
more from the insertion. The distance may be greater rior oblique, passive ductions should be tested and the
with a very loose tendon and less for a tendon that is tightness of the tuck adjusted to a point where the two
not so loose. A hemostat is placed across the tendon sides are equal or the treated side just slightly tighter.
toward the insertion a few millimeters from the suture
and the tendon is cut between the hemostat and

237
Chapter 9

A B

C D

E F

Figure 22
A The superior oblique tendon. D The distal tendon is excised.
B After the loose tendon is elevated, a double arm suture E The tendon is tied securely to the insertion, after adjust-
is placed at the intended amount of resection. ment, if needed, based on superior oblique traction test-
C The tendon is cut distal to the suture and the suture is ing.
passed through the tendon insertion. F The excised tendon.
continued.

238
Surgery of the obliques

G H

Figure 22, contd


G The tendon can be shifted anteriorly if more effect on tor- H The tendon may also be advanced.
sion is required. It may also be tied over a bolster if
adjustment is planned.

239
10
Marginal myotomy:
technique and indications

Historical review
Before the advent of uniform, strong, fine-
gauge sutures with sharp swaged-on needles, partial
or incomplete myotomy was a commonly employed
technique for weakening an extraocular muscle. This
technique has now been superseded by measured
recession, which is the method of choice now for
weakening the rectus muscles in all but a few specif-
ic instances. It is important, however, for the strabis-
mus surgeon to understand the principle of marginal
myotomy and to be familiar with this technique for
use in special cases.
Six types of myotomy that have been
employed for reducing the effect of a rectus muscle
are shown in Figure 1. Three of the procedures (1, 3,
and 5) fail to cut all of the fibers of the muscle and
therefore would not be expected to lengthen the mus-
cle. In contrast, techniques 2, 4, and 6 interrupt all of
the muscle fibers at some point and would be expect-
ed to lengthen the muscle.

Figure 1
1 Central myotomy
2 OConnor triple cut myotomy
3 Incomplete marginal myotomy
4 Overlapping marginal myotomy
5 Multiple incomplete marginal myotomies
6 L-shaped overlapping double marginal myotomy

241
Chapter 10

Figure 2
The preparation for recording the muscle lengthening effect
of a marginal myotomy.

Quantifying the marginal


myotomy
Since, the amount of lengthening produced by
specific marginal myotomy techniques remained
unknown, we attempted to quantify the marginal
myotomy in vitro using freshly prepared rabbit eyes.
These eyes were enucleated after euthenizing the ani-
mals. Between 10 and 20 mm of rectus muscle
remained attached to the globe in the specimens used.
The preparation was mounted as shown in Figure 2
with a weight holding the muscle taut and a camera
situated above to record the lengthening effect of the
myotomies performed. A black silk suture was
placed on the distal muscle as reference to show
change in muscle length after the myotomy. The four
techniques for myotomy shown in Figure 3 were per-
formed five times each and the results were recorded
photographically. Figure 3
Typical results of the experiment are shown in The configurations of the myotomies were as follows:
Figure 4. As would be expected, those myotomy A The muscle was cut from each border 80% of the mus-
techniques that cut across all fibers were effective at cle width with cuts seperated by 30% of the muscle
width.
lengthening the muscle and those not cutting across B The muscle was cut twice at each border for 20% of the
the muscle did not lengthen the muscle significantly. width with cuts seperated by 30% of the msucle width.
This leads us to conclude that if lengthening a rectus C The muscle was cut at its center 80% of the muscle
muscle is more or less equivalent to recessing the width.
D The muscle was cut at its center 80% of the muscle
muscle as a means of weakening the muscle then width and cuts were made at each border 40% of the
only those marginal myotomy techniques that length- muscle width and separated from the central cut by 30%
en the muscle are effective. of the muscle width.

242
Marginal myotomy

A B

C D

Figure 4
A Double 80% overlapping marginal myotomies C A central 80% tenotomy produced lengthening of
seperated by a distance equal to 30% of the 0.06 times the muscles width.
muscles width produced lengthening of 0.9 D Two incomplete marginal myotomies, each
times the muscles preoperative width. including 40% of the muscles width combined
B Multiple, nonoverlapping marginal myotomies with an 80% central tenotomy displaced from the
produced lengthening of 0.03 times the muscles two previous myotomies by 30% of the muscles
width. width, produced lengthening of 0.5 times the
muscles width.

243
Chapter 10

Technique for a double 80%


marginal myotomy
The most useful marginal myotomy in my
experience is the double 80% configuration. To
carry out the procedure, the muscle is first engaged on
a hook, usually after a limbal incision has been per- C
formed to obtain the best exposure. The intermuscu-
lar membrane at the borders of the muscle should be
dissected only far enough to allow placement of the
distal cut. A hemostat is placed across 80% of the
muscle width just behind the insertion and a second
hemostat is placed at the opposite muscle border
across 80% of the muscle with the hemostats seperat-
ed by 30% of the muscles width. (As shown in
Figure 5, I have bent a standard hemostat so that the
jaws are at right angles to make placement of the
instrument easier.) The distal cut is made first. D
If the eye is hyperdeviated the proximal cut
should come down from above to effectively shift the
new insertion down and if the eye is hypodeviated,
just the opposite should be done. If marginal myoto-
my is to be done in a very tight rectus muscle, the
proximal cut can be made with a scalpel which cuts
against the muscle hook behind the muscles inser-
tion. This is done as a safety precaution to guard
against inadvertently cutting through sclera that is
typically thin in such cases.
E

Figure 5
A The muscle is exposed in the usual manner and two
hemostats are each placed 80% of the way across the
muscle (or tendon) from opposite borders. The hemo-
stats are plced 3 or 4 mm apart.
B The posterior hemostat is removed, and scissors are
used to cut across the muscle in the crushed area. By
cutting the muscle in the crushed area, bleeding is kept
to a minimum.
C The hemostat nearer the insertion is removed, and the
B muscle is cut along the crushed area using small snips
with scissors.
D Noticeable lengthening of the muscle will occur. Any
bleeding is controlled with pressure.
E After the distal myotomy has been performed, in a very
tight muscle, a No. 15 Bard Parker blade can be used to
divide the tendon fibers, cutting against the muscle hook.
This can be accomplished with a scraping motion with
the knife blade at nearly right angles to avoid scleral per-
foration.

244
Marginal myotomy

Indications for a marginal


myotomy
Four reasons for doing a marginal myotomy for 3. To weaken a rectus muscle that has at or near
the treatment of strabismus include the following: its insertion an implant, exoplant, or encircling
1. To further weaken a previously operated rectus element used in retinal detachment repair or
muscle that has already been recessed to what for glaucoma filtration.
is considered the maximum amount. 4. To weaken a rectus muscle in a patient who has
2. When combined with a recession to obtain a excessively thin sclera (but a hang loose
double weakening effect while retaining a recession would probably be better).
physiologic arc of contact.

Figure 6
A Marginal myotomy performed on an already recessed lateral rectus muscle provides muscle
lengthening without sacrificing the arc of contact.

245
Chapter 10

Figure 6, contd
B Left hypotropia has occurred after placement of an exoplant and encircling band near the
inferior rectus insertion of the left eye. It has been treated with a double 80% marginal
myotomy of the left inferior rectus.

Other considerations
The concept that myotomy is effective only if with vertical tropia and diplopia.* The average pre-
the muscle is effectively lengthened is being chal- operative deviation was 8 prism diopters. The aver-
lenged. Hertle and associates have demonstrated a age correction at six weeks was 5 prism diopters.
damping effect on nystagmus after detaching and Diplopia was relieved in 70% of patients.
reattaching the four rectus muscles. I continue to believe that for myotomy to be
Alan Scott described graded rectus muscle effective for larger angles and for the longer term,
tenotomy (disinsertion) for treatment of small angle lengthening of the muscle is required. But these
vertical strabismus. He made successive small cuts observations of expert strabismologists must be taken
until the desired results were achieved. Biglan per- seriously when considering the effectiveness of par-
formed a 60% disinsertion of the superior rectus mus- tial myotomy for relieving symptoms of small angle
cle, mostly from the temporal border, in 24 patients strabismus.

* Yim Bin Hye, Biglan A, Cronin TH. Graded partial tenotomy of the vertical rectus muscles for treatment of hypertropia. Trans Amer
Ophthalmol Soc, Vol 102, 2004, pp. 169-176.

246
11
Faden operation
(posterior fixation suture)

Posterior fixation suture


(retroequatorial myopexy,
faden operation)
The modern posterior fixation suture (PFS) rectus muscles with nystagmus on attempted abduc-
was described by Cppers in Germany. It was done tion, and (4) preference for fixation in adduction
initially in the United States in 1975 after being intro- while the head turns in the opposite direction, with or
duced by Mhlendyck. The procedure has been pop- without occlusion of the opposite eye, or for fixation
ularly called the faden operation. Faden in German with asymmetric convergence while the head remains
means suture or string. However, more descriptive straight. Most of these characteristics are shared by
names, posterior fixation suture or retroequatorial the Ciancia syndrome patients as a manifestation of
myopexy more appropriately describe the procedure. congenital esotropia.
The aim of the posterior fixation suture is to The posterior fixation suture has also been
shift the effective insertion of a rectus muscle poste- used on the superior rectus muscles to treat dissociat-
riorly employing a principle that was described first ed vertical deviation done with or without recession
by Peters more than forty years before. This posteri- of the superior rectus. However, this procedure did
or shift of the muscles insertion theoretically reduces not seem to have sufficient power and has been
the effect of the muscle only in its field of action. The replaced by large recession of the superior recti, and
posterior fixation itself is designed to have little if any in selected cases anterior transposition of the inferior
effect in the primary position. However, if the mus- obliques and, in persistent cases, inferior rectus resec-
cle posterior to the suture is on a stretch while the tion.
suture is placed, redundant muscle between the origin Another indication for the PFS, and in my
and the fixation suture may effect the muscles action opinion the very best and most useful, is to weaken
in primary position. This may be the reason for the sound yoke of an underacting muscle in order to
reports saying that PFS done on the medial recti are create a secondary deviation which both boosts the
effective in reducing an esodeviation (Figure 1). action of the weak muscle and slightly limits the
The PFS has no effect on the initiation of eye action of the sound muscle. This is what I call a laud-
movement or on the behavior of eye movement in the able secondary deviation. Here is an example: A
field opposite the muscle having the PFS. The prin- patient has a weak left lateral rectus muscle with lim-
cipal effects of the PFS are to somewhat limit the ited abduction of the left eye and diplopia in levover-
movement of the eye in the field of action of the mus- sion beginning just a few degrees beyond the midline.
cle treated and to cause increased innervation to this With a posterior fixation suture placed on the right
muscle and its yoke by Herings law as the eye medial rectus two things happen. First, the right
attempts to move in the field of muscle with the PFS. medial rectus works harder to move toward levover-
At the outset, the indication for the PFS was to sion and therefore sends more innervation to the left
treat the nystagmus blockage syndrome. This condi- lateral rectus by Herrings law (a secondary devia-
tion has been said to be characterized by: (1) mani- tion). Second, the right eye cannot go as far in levo-
fest nystagmus damped by convergence, (2) variable version because of the PFS and therefore is less like-
angle esotropia, (3) pseudoparalysis of both lateral ly to exceed the excursion of the left eye in usual

247
Chapter 11

A B

C
D

Figure 1
A The eye is shown from above the medial rectus on top. D As the eye moves back to the primary position, the medi-
B With the eye in abduction, the medial rectus is stretched. al rectus behind the suture is loose or redundant. This
C The posterior fixation suture is placed with the medial would theoretically weaken the effect of the medial rectus
rectus on the stretch. in the primary and thereby reduce the esodeviation.

visual activity thereby reducing the likelihood of less important than down gaze including the reading
diplopia (Figure 2). position (except in certain exceptions) this procedure
Although not treating yoke muscles, a PFS is less likely to be indicated for the superior rectus
may be placed on the normally acting inferior rectus (Figure 3).
in case of weakness of the other inferior rectus. In I have used a posterior fixation on the four hor-
this example, there is no secondary deviation effect, izontal rectus muscles as a means of treating nystag-
only the pure limitation of movement or mus. The few cases that I treated were not success-
pseudoparalysis of the sound muscle treated with ful. Recession of the four horizontal rectus muscles
the PFS. This would result in the two eyes being has been effective for treatment of nystagmus, and
more nearly matched in down gaze thereby avoiding Hertle has reported success with simple disinsertion
or at least reducing diplopia. Because far up gaze is and reinsertion of the muscles.

248
Faden operation (posterior fixation suture)

Figure 2
A The muscle is secured to underlying sclera at or just C The new effective insertion creates a reduced lever arm
behind the equator. for the muscle. This reduced lever arm results in the
B The extent of rotation of the eyeball is restricted by the need for increased innervation for the muscle to achieve
suture compared to movement without the suture in its full (though reduced) rotation.
place.

249
Chapter 11

OD OS

OD OS

OD OS

OD OS

Figure 3
A The eyes are aligned in the primary position. D With a posterior fixation suture placed on the normal left
B The right eye has deficient depression from a right inferior rectus, the movement downward in this eye is
inferior rectus paresis. somewhat limited making it more nearly match the right
C In far down gaze, the left eye moves normally, resulting eye. The yoke of the left inferior rectus, the right
in an increasing right hypertropia and diplopia in the superior oblique, a depressor of the right eye, would also
reading position. receive more innervation to down gaze.

250
Faden operation (posterior fixation suture)

Placement of the posterior


fixation suture after detaching
the muscle
A To be effective, it has been said that the poste-
rior fixation suture should be placed at or just behind
the equator. However, recent description of the mus-
cle pulleys has led to a concept that the PFS need not
be placed behind the equator to be effective. In either
case, the placement of the suture can be challenging
and requires good exposure (Figure 4).

Placement of the posterior


fixation suture without
detaching the muscle
A posterior fixation suture may be placed with-
out recession of the muscle in cases where no effect is
needed in the primary position. This is usually done
with placement of sutures at each muscle border
(Figure 5).

B
A

Figure 4
A The rectus muscle may be tagged with sutures at the
insertion and detached. Two sutures are then placed in
sclera at or just behind the equator to secure the muscle
Figure 5
at the borders. Non-absorbable suture, 5-0 or 6-0 is A After obtaining adequate exposure, the muscle is retract-
used. ed and a 5-0 or 6-0 non-absorbable suture is placed at
B The posterior fixation suture sutures are tied securely. the muscle border at or just behind the equator. A limbal
C The muscle is reattached at the insertion. incision is a good choice to gain best exposure. The flap
is being held with forceps. Better exposure is obtained
with a Barbie retractor.
B After placement of the sutures, the muscle looks relative-
ly undisturbed. (An optional single central suture is
shown)

251
Chapter 11

Reinforced posterior fixation Combined posterior fixation


suture and recession
The purpose of the posterior fixation suture is When recession of a rectus muscle is
to produce a firm adherence of the undersurface of the combined with placement of a posterior fixation
muscle to sclera beneath and thereby create a new suture, the recession should be carried out before the
effective insertion for the muscle. To accomplish posterior fixation suture is placed. This ensures that
this, some surgeons prefer to make several passes the recession will be effective. If this were not done,
through sclera and muscle. When multiple suture redundant rectus muscle would be distal to the
passes are employed, the suture is placed in the usual posterior fixation suture that would be the new
manner except that two or three bites are taken in effective insertion, reducing or nullifying the
sclera and after each of these bites, the suture is recession effect (Figure 7).
brought through the muscle. The potential problem
with this is that the chance of scleral perforation is
increased. This problem is accentuated by the fact
that needles usually found on 5-0 or 6-0 non-
absorbable sutures are often heavier cutting needles
that increase the likelihood of inadvertent scleral per-
foration. To avoid this, the surgeon should exercise
great care in placing the needle in sclera (Figure 6).

A Figure 7
Recession has been carried out and then the posterior fixa-
tion suture is placed. This means that the length of muscle
between the PFS and the muscle origin is longer, thereby
achieving both the recession effect and the posterior fixa-
tion suture effect.

Adjustable faden
Alan Scott has suggested a novel method for creating
a posterior fixation suture in a way that is potentially
adjustable. To do this, a double arm suture is placed
in the muscle at about the point where a posterior fix-
ation suture would be placed. The muscle distal to
this suture is then excised! This means that the mus-
cle is not shorter, it just inserts closer to the origin as
B with the posterior fixation suture. The sutures are
then brought through the muscles original insertion
and a suitable temporary knot is placed. The theory
behind this technique is that the muscle distal to the
suture placement of the posterior fixation suture is
probably irrelevant anyway (Figure 8).

Figure 6
A For a reinforced posterior fixation suture, the belly of
the muscle is elevated and two or three suture passes
go through sclera and muscle.
B After the identical suture placement is carried out on the
other border of the muscle, the sutures are tied securely.

252
Faden operation (posterior fixation suture)

A
B

Figure 8
A A double arm suture is placed 12 to 14 mm from the C Suture ends are brought through the original insertion.
insertion, or at the point on the muscle where the posteri- D A knot that can be released for adjustment and tied
or fixation suture would be placed. securely later is placed.
B The muscle/tendon distal to the suture is excised.

253
12
Adjustable sutures:
techniques for restrictions

Overview
Adjustable sutures were used commonly in the muscle securely to sclera at a predetermined point
early years of strabismus surgery. They were used out based on patient history, work-up, and intra-operative
of necessity rather than choice. Sutures were coarse findings.
and needles were heavy with the result that muscles I use adjustable sutures in fewer than 10% of
could not be attached to sclera either accurately or cases. This is actually an increase from about 3%
safely. Sutures were anchored in conjunctiva and earlier. This increase is due to the use of the tandem
Tenons with the muscle lying on but not attached to adjustable that actually requires adjustment only 25%
sclera. Adjustments may not have been precise in of the time. When adjustable sutures are used they are
these patients, but were necessary to obtain the best limited to older children and adults with conditions
possible results. including: previous unsuccessful surgery, thyroid
When catgut sutures with swaged-on needles myopathy, diplopia after successful cataract surgery,
became the standard for strabismus surgery, strabismus after trauma, restrictive strabismus, etc.
adjustable sutures were no longer employed. These For treatment with an adjustable suture, a muscle
sutures had the advantage of being absorbable and the should have contractile power to rotate the eye and
needles were finer making for more accurate surgery. should be working against an antagonist muscle
The disadvantage of catgut sutures was that they capable of relaxing.
broke easily making them unsuitable for the
adjustable technique. Actually a suture broke once in Technique for the adjustable
almost every case during the course of surgery, suture
especially when an assistant was allowed to tie a knot.
There are several effective techniques for
During the 1960s when I started doing strabismus
placement of an adjustable suture. Regardless of the
surgery adjustable sutures were neither done widely
technique used, the following principles remain
nor talked about.
constant.
Adjustable sutures were re-discovered and
1. The muscle is secured with suture that is
popularized by Jampolsky after synthetic absorbable
sufficiently strong to withstand postoperative
sutures became available in the 1970s. This
manipulation.
technique is currently used widely for treatment of a
2. The muscle is attached to the globe usually at
wide variety of strabismus conditions. Their use,
the muscles insertion stump in a hang loose
however, is not universal. Some surgeons use
manner.
adjustable sutures for nearly every case stating that
3. The suture anchoring the muscle is secured in
better results are achieved if a second chance is
a way that it can be easily loosened and then
available or saying that better results can be achieved
re-tied at the time of adjustment.
if the postoperative alignment can be determined with
4. The suture is able to slide through the site of
the patient awake and cooperating. Other surgeons
attachment to the globe allowing the muscle
use them either sparingly or not at all. The belief
to slide back or be pulled forward.
among these surgeons is that in most (all) cases more
precise surgery can be accomplished by attaching the

255
Chapter 12

A B

C D

E
F

Figure 1
A The muscle is exposed. E The sutures are brought through the muscles stump.
B The suture is placed 1 to 1.5 mm from the insertion (a F If a bolster is used, the suture is brought through after
handle-suture as shown in Figure 2 is placed in all putting the needles through conjunctiva to produce a
cases). conjunctival recession (as shown) or through conjunctiva
C The suture is secured with a central bite which is tied overlying the stump.
and locking loops are placed at the borders. G The suture is tied over the bolster (if used) or on con-
D If the muscle is tight, it is cut from the globe with a junctiva.
scalpel, cutting against a muscle; otherwise scissors are
used.

256
Adjustable sutures: techniques for restrictions

5. The incision in conjunctiva should be made only suggest leaving the eye in the same alignment
so that the surgeon can access the suture at the that is preferred when standard surgery is performed;
time of adjustment and then close the that is, a slight overcorrection.
conjunctiva satisfactorily with the patient A handle of 6-0 Vicryl placed in sclera at the
awake. limbus as a means of grasping and manipulating the
6. The use of adjustable sutures are effectively globe during adjustment is shown in Figure 2. The
limited to the rectus muscles (Figure 1). handle must be exposed at the conclusion of surgery
In every case of adjustable suture, a so-called regardless of which type of incision has been used. A
handle suture is placed in superficial sclera usually forceps grasping the handle allows relatively easy
near the limbus. This is used for stabilizing the eye, rotation and stabilization of the globe during surgery
securing the muscle, and in rotating it at the time of and during adjustment. A three-cornered limbal
adjustment. The suture can be temporarily tied with incision may be used that can be taken down at the
a bow know, a slip knot, or a noose like cinch knot time of adjustment and repaired when the adjustment
that can be secured tightly or loosened to slide up and is completed. A sliding knot over the sutures
down the suture as needed during adjustment. suspending the muscle can be helpful during
Use of an adjustable suture begs the question, adjustment. The sliding knot is secured at surgery
Where should the eyes be placed at the time of when the muscle is at the intended position and the
adjustment?. While there is no reliable answer to suture ends are tied. At adjustment, the knot may be
this question, I tend to leave the eyes in the alignment loosened and slid toward the cornea while the patient
I would like to achieve at the same period looks in the opposite direction as the globe is
postoperatively if the muscle had been firmly stabilized with the handle suture if the recession
attached to sclera at the time of operation. Patients effect is to be increased. If the recession effect is to
with postoperative diplopia are adjusted to a diplopia- be lessened, the suture holding the muscle is pulled
free position. Non-fusing exotropic patients are left up and the slip knot is slid toward the muscle. When
straight or slightly exotropic; non-fusing esotropic a cul-de-sac incision is used, a handle suture is placed
patients are left straight or slightly esotropic. at the upper insertion in case of an inferiorly placed
Because I never use an adjustable suture in a patient incision. For adjustment, this suture is pulled up to
treated surgically for intermittent esotropia, I can center the incision over the muscle stump.

A B

Figure 2
A The handle suture. B The suture ends must be retrievable.
continued.

257
Chapter 12

C D

E F

G H

Figure 2, contd
C The handle suture stabilizes the globe during adjustment. G The muscle is advanced or it drops back sliding through
D A three cornered limbal incision may be used. the slip knot which is tightened when the muscle is in the
E The sutures may be secured with a slip knot. intended position.
F The amount of hang back can be measured. H Both the handle suture and the adjustable suture are led
out through the cul-de-sac (inferior) incision shown.
I The handle suture lifts the incision over the muscle inser-
tion.

258
Adjustable sutures: techniques for restrictions

Tandem adjustable suture


A useful modification of the adjustable the second or tandem suture is simply tied tighter
suture is the tandem adjustable suture. It combines without the risk of the muscle slipping back and the
the hang back suture with the adjustable suture possibility of the surgeon losing track of where the
(Figure 3). Using this technique a static hang back muscle is at the start of adjustment. If the muscle
suture is placed putting the eye at the position the needs to be let back farther, the first or hang back
surgeon thinks will be most likely to achieve the suture is cut and the second tandem suture is used in
desired alignment. If that is the case, a stable the usual manner for an adjustable suture. In my
situation prevails and there is no need to manipulate experience, the tandem suture needs adjustment less
the suture in the process of re-tying, and securing as than 25% of the time. This makes the tandem suture,
would be required if a single suture capable of in my experience, the most convenient technique to
adjustment were used. If the muscle is too far back, use when an adjustable suture is called for.

A B

Figure 3
A Two 6-0 vicryl sutures are placed 1.5 and 2.5 mm from C The incision is closed with the adjustable suture ends
disinsertion. exposed. If no adjustment is needed the adjustable
B The muscle is detached and the proximal (nearer the sutures are cut.
insertion) sutures pass through the stump producing a continued.
hang loose recession, placing the muscle where the
surgeon thinks best. The distal sutures are brought
through the stump outside the first sutures - they are left
untied.

259
Chapter 12

Figure 3, contd
D If at adjustment the muscle is to be let back the first E If the muscle is to be adjusted closer to the insertion, it is
suture is cut and the muscle is adjusted using the sec- simply pulled up with the adjustable suture. The first
ond suture. suture is redundant.

Adjustable suture
considerations
Important considerations for the adjustable the eye appropriately and then carrying out cover test-
suture technique are ease of adjustment, accuracy of ing. If the patient is able to respond, I ask whether the
final alignment, and patient comfort during and after patient sees two and by careful adjustment work to
surgery and during adjustment. Enthusiasm for use of eliminate the diplopia. If general anesthesia is used,
adjustable recession and resection varies widely I merely attempt to center the eye and carry out the
among even the most experienced surgeons. Some adjustment later. The suture may be adjusted on the
never use this type of surgery stating that the tech- day of surgery, or in the recovery room an hour or
nique is absolutely unnecessary and that excellent more after surgery if the alignment is significantly
results can be obtained without using adjustable different than intended. The most common and most
sutures. Other equally experienced and competent productive time for adjustment is approximately 24
surgeons use adjustable sutures in every patient who hours after surgery, either at the bedside for those
will cooperate for adjustment with few exceptions. patients who are admitted to the hospital or in the
The majority of surgeons probably occupy the middle clinic for outpatients. Topical anesthesia with
ground, using the adjustable suture technique only in proparacaine hydrochloride, tetracaine, or 5%
selected cases. I perform adjustable suture surgery on Xylocaine may be used. A lid speculum is helpful to
less than 10% of adult patients undergoing strabismus give exposure while picking up the suture ends.
surgery. Tying forceps are used to grasp the suture holding the
As with the use of adjustable sutures, timing of muscle and a fine utility forceps is used to grasp the
adjustment varies widely among surgeons. When a handle suture. Scissors are used to trim the suture
patient has topical or perilimbal anesthesia with min- ends after the adjustment has been completed. One
imum sedation, I prefer to carry out the adjustment surgeon I know claims to be able to adjust a muscle as
while the patient is on the operating table, positioning late as 10 days after surgery. Although I believe her,

260
Adjustable sutures: techniques for restrictions

I would not attempt an adjustment that long after sur- When reoperating a patient who has undergone a
gery. From experience with a few cases of early reop- previous conjunctival recession, it is necessary to
eration, I believe that a fairly firm myoscleral union is enter sub-anterior Tenon's space at the point where
formed in just a few days. Rather vigorous manipu- conjunctiva had been recessed. The sclera in the area
lation of the globe is required to accomplish late mus- of conjunctival recession becomes re-epithelialized
cle adjustment. As with surgery performed with top- with a thin layer that adheres tightly to underlying
ical or local anesthesia, the pain during adjustment sclera. It should not be disturbed. Patients are usual-
comes mainly from traction on the muscle and is deep ly comfortable after conjunctival recession.
in the orbit. This can cause syncope and nausea. For Ointment is used twice a day after surgery (switching
adjustment it is a good idea to either have the patient to drops in the morning if ointment causes blur) and
lying down or have a bed available nearby. The no patching is necessary. It is also important to
patient may take a mild oral analgesic a few hours remember that conjunctiva becomes extremely thin
before the adjustment to make them more comfort- and friable in older patients. Even some patients in
able. their 20's may have very thin conjunctiva. Nearly all
patients 30 years and older have very thin conjuncti-
Conjunctival recession va. Therefore, it is impractical to attempt a cul-de-sac
incision in an older patient unless the surgeon has
When the conjunctiva is recessed because of
inspected the conjunctiva and has determined that it
tightness that restricts free movement of the eye, it is
could withstand the necessary manipulation.
usually necessary to move the limbal margin of the
Eyes with longstanding esotropia usually have a
conjunctiva back 5 to 7 mm or to the insertion site of
foreshortened conjunctiva that restricts abduction. A
the recessed muscle. In the rare case where conjunc-
limbal incision is made in the usual manner encom-
tival recession is performed without recessing the rec-
passing approximately 2 to 3 clock hours centered
tus muscle, the limbal margin is moved to a point just
over the muscle's insertion with radial relaxing inci-
covering the insertion of the muscle. When the con-
sion approximately 10 mm long. For closure with
junctiva is severely scarred it may be excised and the
recession, conjunctiva-Tenon's is sutured to sclera
cut edge of conjunctiva attached to underlying sclera.
with three interrupted 8-0 Vicryl sutures used. With
This step may be carried out medially as far as the
the conjunctiva sutured in place, the bare sclera is left
plica semilunaris. Because the medial conjunctiva
to re-epithelialize in a day or so.
containing the plica semilunaris and caruncle has
With a severely scarred conjunctiva, the entire
more tissue and because the medial recti are the most
conjunctival flap may be excised and the cut end of
frequently operated extraocular muscles, medial con-
conjunctiva sutured to underlying sclera and the
junctiva is the area most frequently in need of revi-
relaxing incision sites sutured to adjacent conjuncti-
sion. In contrast to reddened scars of the conjunctiva,
va-Tenon's. When medial scarring is extensive, the
clear subconjunctival cysts which occur occasionally
medial conjunctiva can be excised as far medially as
after eye muscle surgery can be removed, sometimes
the plica semilunaris. The plica is then sutured direct-
intact, without the need to recess the conjunctiva pro-
ly to underlying sclera far medially (Figure 4).
vided the overlying conjunctiva remains elastic.

A B

Figure 4
A Tight, scarred conjunctiva B For conjunctival recession A is attached to A1 and C to
C1. A third suture is placed in the center.
continued.

261
Chapter 12

C D

F G

H I

Figure 4, contd E Scarred medial conjunctiva


C Conjunctival recession sutures are placed. G and the cut edges of conjunctiva sutured to sclera.
D The conjunctival recession is completed. H In extreme cases of conjunctiva scarring,
F Very thick conjunctiva can be excised, I Pratt-Johnson has excised conjunctiva to the caruncle.

262
Adjustable sutures: techniques for restrictions

Traction sutures
When the surgeon is concerned that postopera- taken at the 6 o'clock limbus position and the 4-0 silk
tive adhesions may cause the globe to remain fixed in sutures are taken out through the upper tarsus and tied
an undesirable position, traction suture placement over a rubber or silicone peg. To fix the eye is deor-
may be used. The eye should always be placed in a sumduction, the two bites are taken at the 12 o'clock
position opposite the undesirable fixation. A chroni- position with 4-0 silk sutures, and the sutures are
cally esodeviated eye with restricted abduction brought out through the lower tarsus and tied over a
should be fixed in abduction, a Brown syndrome rubber or silicone peg.
should be fixed in adduction and sursumduction, and Some surgeons prefer to anchor traction
so on. sutures through the tendinous insertion of the rectus
In Figure 5, the right eye is to be placed in muscles. The attachment to the globe is more secure
forced abduction. Two scleral bites are taken near the with this technique. The traction sutures are placed at
nasal limbus with 4-0 silk sutures. The sutures are the insertion of the superior and inferior rectus before
brought out through the upper tarsus and tied over a fixing the eye in abduction or adduction. Traction
rubber or silicone peg with the eye in several degrees sutures are placed at the insertion of the horizontal
of abduction. The sutures are removed in 5 to 7 days. recti to fix the eye in sursumduction or deorsumduc-
Because the eye is rotated, corneal contact by the tion. The right eye is fixed in abduction and the
suture is kept to a minimum. To place the eye in sutures are brought out through the temporal aspect of
forced adduction, suture placement is reversed. To the upper lid, fixing the eye in the abducted position.
fix the eye in sursumduction, two scleral bites are

A B

C D

Figure 5
A A 4-0 black silk suture is secured to sclera. D The eye anchored in depression.
B The eye anchored in adduction. E The eye anchored in elevation and adduction with two
C The eye anchored in elevation. traction sutures placed in the insertion of the superior
and inferior rectus.

263
13
Muscle transposition
procedures

Overview
When an extraocular muscle is paralyzed it has ist, the mechanical restrictions must be eliminated
lost the ability to contract. The usual strengthening before carrying out the extraocular muscle transfer.
techniques such as resection, advancement, or tuck This release of mechanical restrictions can be done
(actually shortening procedures) do not restore the with surgery or to some extent by chemodenervation
muscles potential for normal ocular rotation. A new, with Botox.
more favorable, static position of the globe may be Extraocular muscle transfer achieves a change
accomplished after a large recession-resection proce- in the mechanics of a given muscle but innervation to
dure, but movement in the field of action of the para- this muscle remains the same as preoperatively, and
lyzed muscle is not accomplished. To remedy this, the muscle continues to obey Herings law. A suc-
Hummelsheim in 1907 devised a procedure to trans- cessful extraocular muscle transfer procedure has
fer part of the action of the superior and inferior rec- most of its effect in changing alignment in the pri-
tus muscles to the field of action of the lateral rectus mary position with only a limited effect in the field of
muscle in cases of sixth nerve palsy. action of the paralyzed muscle. This movement may
This procedure has undergone numerous mod- be due to a spring load effect created by the trans-
ifications in the last century, but most retain the basic ferred muscles and activated when the antagonist
principle of the technique as introduced by relaxes according to Sherringtons law during
Hummelsheim. The principle is that action of mus- attempts to look in the field of action of the paralyzed
cles that are normally antagonists are transferred to muscle. Some patients with acquired sixth nerve
the field of action of the muscle lying between these palsy and also some with acquired vertical rectus
antagonists. For example, the superior and inferior palsy can achieve expanded areas of diplopia-free
rectus muscles are transferred to a point adjacent to vision after muscle transfer, and others just look bet-
the lateral rectus muscle in sixth nerve palsy or to the ter in the primary position while retaining large areas
medial rectus in case of medial rectus palsy. The hor- of diplopia.
izontal recti are likewise shifted adjacent to the supe-
rior rectus in superior rectus palsy and to the inferior A review of muscle
rectus when this muscle is paralyzed. transposition procedures
Muscle transposition may be indicated in any In Hummelsheim's original transplant proce-
case where paralysis of a muscle is associated with an dure, the lateral halves of the tendons of the superior
unacceptable deviation in the primary position and/or and inferior rectus muscles are attached to the tendon
bothersome diplopia. This can occur in unilateral or of the lateral rectus (Figure 1A). In O'Connor's mod-
bilateral sixth nerve palsy, double elevator palsy, infe- ification of the Hummelsheim procedure the entire
rior rectus palsy, with an irretrievable lost muscle, tendons of the superior and inferior rectus muscles
and other causes. It should be emphasized that in are sutured to the sclera adjacent to the insertion of
cases of extraocular muscle paralysis, especially the lateral rectus and a cinch is performed on the lat-
those of longstanding, mechanical restriction can be eral rectus (Figure 1B). In a further modification of
present in the antagonist. When paralysis and O'Connor's technique the nasal halves of the superior
mechanical restrictions limiting free movement coex-

265
Chapter 13

A B

C D

E F

Figure 1 Muscle transposition procedures


A Hummelsheim E Peter
B OConnor F Hildreth
C Modified OConnor G Schillinger
D Wiener continued.

266
Muscle transposition procedures

H I

J K

Figure 1, contd Muscle transposition procedures


H Beren-Girard J Uribe
I Jensen K Knapp

and inferior rectus tendons are passed beneath the (Figure 1H). In Jensen's technique the superior rec-
temporal halves of the insertions and attached to the tus, inferior rectus, and lateral rectus muscles are split
sclera adjacent to the lateral rectus tendon (Figure along their long axes. The lateral half of the superior
1C). In Wiener's procedure the paralyzed lateral rec- rectus is joined to the superior half of the lateral rec-
tus is transected and the proximal tendon is split and tus and the inferior half of the lateral rectus and the
joined to the adjacent superior and inferior rectus lateral half of the inferior rectus are joined in a simi-
muscles (Figure 1D). In Peter's procedure for third lar fashion with nonabsorbable sutures. The medial
nerve palsy, the trochlea is fractured and a shortened rectus may or may not be recessed. This procedure
superior oblique tendon is sutured to the sclera near performed on appropriate muscles also has been sug-
the insertion of the medial rectus (Figure 1E). In gested for double elevator palsy, medial rectus palsy,
Hildreth's procedure the entire tendons of the superi- and double depressor palsy (Figure 1I). In Uribe's
or and inferior rectus muscles are joined with nonab- technique the medial rectus is recessed, the lateral
sorbable suture (Figure 1F). rectus resected, and the entire tendon of the superior
In Schillinger's procedure the entire tendons of and inferior rectus muscles sutured to the sclera adja-
the superior and inferior rectus muscles are sutured to cent to the resected lateral rectus insertion (Figure
the sclera near the insertion of the lateral rectus 1J). In Knapp's technique for double elevator palsy
(Figure 1G). In Beren's and Girard's technique the the entire tendon of the medial and lateral rectus mus-
medial rectus is recessed, the lateral rectus resected, cle is shifted and sutured to the sclera adjacent to the
and both superior and inferior rectus muscles shifted insertion of the superior rectus. The inferior rectus
one-half width temporally, with the temporal half of may also be recessed. This full tendon transfer may
each muscle sutured to the resected lateral rectus be used for any of the rectus muscles (Figure 1K).

267
Chapter 13

Rectus muscle transfer


Transfer of antagonist rectus muscles to a posi-
tion near the insertion of the paralyzed rectus muscle
lying between is the technique currently used for
most extraocular muscle transposition. This can be A
carried out for any of the rectus muscles. It is done
most frequently shifting the superior and inferior rec-
tus muscles to the lateral rectus muscle for sixth nerve
paralysis to offset, at least in part, absence of abduc-
tion. This type of transfer is also used for the superi-
or, inferior and less often for the medial rectus mus-
cle when function is lost because of paralysis or phys-
ical damage to the muscle.
The entire muscle or just one half of the mus-
cle is shifted in this procedure. Full tendon transfer is
more powerful, but it sacrifices both anterior ciliary
arteries in the transferred muscle. Shifting only one
half of the muscle spares one anterior ciliary artery in B
each of the transferred muscles if care is exercised.
To add more power to the transfer, the border of the
transferred muscle can be sutured to the paralyzed
muscle as described by Foster or adjacent to it as
described by Buckley. In addition, the antagonist can
be weakened by recession at the time of surgery or by
Botox a week or two before surgery or sometimes
after.
This surgery is begun with a limbal incision
that extends for 180 degrees (Figure 2). The muscles
to be transferred are isolated on a muscle hook and
the intermuscular membrane on both borders is freed
from the muscle border fifteen or more millimeters
from the insertion. Either the entire muscle is hooked
and one or two sutures are placed 1or 2 mm behind
the insertion or the muscle is split making sure that
the anterior ciliary artery in the half of the muscle
remaining remains undisturbed. Suture(s) are then C
placed in the muscle half. The muscles are detached
and reattached to sclera just touching the paralyzed
muscle between with the transferred insertion placed
concentric with the limbus.
The antagonist rectus may be recessed leaving
only the remaining anterior ciliary vessel(s) in the
paralyzed muscle if a full tendon transfer has been
done, or at least three if a half tendon transfer has
been performed. In selected cases the antagonist may
be temporarily paralyzed at surgery by injecting up to
5 units of Botox.

Figure 2
A A limbal incision is made for 180 degrees. This proce-
dure is shown for transfer of the superior and inferior
rectus to the lateral rectus, but it may be done for any of
the rectus muscles.
B The three rectus muscles are exposed.
C For a full tendon transfer, one or two sutures are placed
1 or 2 mm behind the insertion of the muscle to be trans-
ferred using one double arm or two single arm sutures.
continued.

268
Muscle transposition procedures

D E

F G

Figure 2, contd
D After detaching the muscles, they are reattached to scle- F For a more powerful muscle transfer, the borders of the
ra concentric with the limbus with one border of the mus- muscle can be joined with a non-absorbable suture 8 mm
cle just touching the edge of the insertion of the para- behind the insertion of the paralyzed muscle, or these
lyzed muscle. transferred edges can be sutured to sclera adjacent to
E For a half muscle transfer, the muscle is split with a mus- the paralyzed muscle. This can be done with a full or a
cle hook and is separated backward for 15 mm. A suture half tendon transfer.
is placed in the half of the muscle to be transferred, the G The antagonist rectus may be recessed.
muscle is detached and reattached just touching the H Botox may be injected in the antagonist.
edge of the insertion of the paralyzed muscle. Care
should be exercised to spare the remaining ciliary artery.

269
Chapter 13

Scleral augmented muscle-


tendon transfer
In cases of strabismus where a rectus muscle is missing medial or lateral rectus muscle. I have also
missing because of trauma, previous surgery, or con- threaded 5-0 nonabsorbable suture in the scleral strip
genital absence, a traditional muscle transfer proce- for added strength.
dure cannot be performed. In cases such as this A 54-year-old woman with a large left
where the antagonist is tight, it must be recessed. exotropia who had undergone two previous proce-
This in turn rules out a full tendon transfer of the two dures for esotropia is shown (Figure 3). Both eyes
remaining rectus muscles because of the chance of had been operated on at each procedure. The last
anterior segment ischemia after detachment of all the operation had been performed when the patient was
rectus muscles of a given eye. A Jensen procedure 17 years old and resulted in this large exotropia. The
cannot be performed because the absence of a rectus left eye could not move even to the midline, passive
muscle rules out rectus muscle union. With the ductions were severely restricted in the direction of
advent of Botox the tight antagonist can be injected, adduction, and no generated muscle force was meas-
but this neurotoxin treatment cannot be expected to ured in attempted adduction. At surgery, no medial
release tightness associated with a chronic deviation rectus was found. A 1.5 x 100 mm strip of glycerin
caused by secondary structural changes in the muscle, preserved human sclera was prepared. The middle of
in the conjunctiva, anterior Tenon's capsule, and the scleral strip was sutured at the point where the
intermuscular membrane (posterior Tenon's capsule), medial rectus muscle would have inserted. One end
and it cannot change movement in the field of action of the scleral strip was passed beneath the medial half
of the missing muscle. of the superior rectus that had been split along its long
The scleral augmented muscle-tendon transfer axis (Figure 4). The inferior rectus muscle was also
avoids the condition that could cause anterior seg- split along its long axis and the other end of the scle-
ment ischemia; that is, removal of all remaining rec- ral strip was placed around the medial half of the infe-
tus muscles with their anterior ciliary arteries - and at rior rectus. One anterior ciliary artery was left undis-
the same time allows completion of the equivalent of turbed in both the superior and inferior rectus muscle.
a Jensen procedure. The scleral augmented muscle- The tight lateral rectus was then recessed. After this,
tendon transfer is accomplished by first recessing the the scleral strips were drawn toward each other,
tight antagonist and then suturing a 1.5 mm wide pulling the split halves of the rectus muscles toward
band of preserved sclera to host sclera approximately the area on the globe usually occupied by the inser-
at the site of the normal insertion of the missing mus- tion of the missing muscle. The scleral strip ends
cle. The long (approximately 100 mm) strip of scle- were sutured together with 5-0 nonabsorbable suture
ra is threaded through the two adjacent rectus muscles with the eye in a slightly overcorrected position. The
that are divided along their long axes. The ends of the thin scleral strip may be passed beneath the split mus-
scleral strip are then drawn together, pulling the mus- cle or over the outer surface of the split muscle, as
cle slips toward the area of the missing muscle. shown here, without altering the outcome of the pro-
When the eye is slightly past the midline, the scleral cedure. A satisfactory primary position alignment
strip ends are joined with nonabsorbable 5-0 suture. after scleral augmented extraocular muscle transfer
We have used this procedure successfully in cases was accomplished. Adduction was achieved but the
where a large horizontal strabismus was caused by a eye could move only a few degrees (Figure 5).

Figure 3
A large exotropia in a patient who at surgery was found to have no left medial rectus.

270
Muscle transposition procedures

A B

Figure 4
A The scleral strip is sutured 5.5 mm from the limbus, the B The scleral strip pulls the vertical recti toward the empty
vertical rectus is split, and the scleral strip is passed medial rectus insertion site. The antagonist is recessed.
through the split muscle. C The ends of the scleral strip are joined with non-
absorbable sutures.

Figure 5
Postoperative alignment is good.

271
Chapter 13

Knapp procedure
The Knapp procedure remains a popular tech- The procedure starts with a large limbal inci-
nique for muscle transposition. It is useful for treat- sion or equivalent (Figure 6). The medial and lateral
ing double elevator palsy especially when there is no rectus muscles are secured with two single arm or one
mechanical restriction to elevation. This technique double arm suture, are detached and reattached at the
employs upward shift of the medial and lateral rectus corner of the superior rectus insertion. Subtle differ-
muscles to a point adjacent to the corners of the inser- ences in the alignment of the insertions of the trans-
tion of the superior rectus muscle. It differs from ferred muscles can be employed to modify both the
other transpositions of the full tendon in that the line horizontal and the vertical pull. In theory the higher
of insertion of the transposed muscles is more or less the placement of the new insertions, the more the
parallel to the borders of the superior rectus. This is upward pull on the eye. In addition, location of the
in contrast to those full tendon transfer procedures placement of the new insertions, especially that of the
that have the new insertion of the transferred muscles lateral rectus muscle can affect the horizontal align-
concentric with the limbus. This may make a differ- ment. I produced a large transient exodeviation in
ence in the pull of the transposed muscle, especially one patient after doing this procedure for double ele-
if the concentric insertion transfer is reinforced with a vator palsy. The conjunctiva is closed in the usual
suture joining the transposed muscle to the paralyzed manner.
muscle 8 mm posterior to the insertion.

Figure 6
A A large limbal incision ( or equivalent) is made. C The incision is closed with 8-0 absorbable suture.
B After placing one or two sutures in the medial and lateral
rectus muscles, these muscles are detached and reat-
tached adjacent to the border of the superior rectus mus-
cle.

272
Muscle transposition procedures

Superior oblique tendon


transfer
Superior oblique tendon transfer can be a use-
ful procedure in cases of complete third nerve palsy.
Theoretically the transferred muscle should act more
like a tether holding the eye in adduction than as a A
functioning rotator. Nevertheless, the improvement
in alignment that results from this procedure resulting
in improvement in the patient's appearance has kept
superior oblique tendon transfer in the strabismus sur-
geon's armamentarium. In the past I have attempted
to fracture the trochlea in order to bring the superior
oblique tendon forward from the apex of the orbit
producing a more physiologic pull for adduction.
However, this resulted too often in either inability to
fracture a tough trochlea, cutting the superior oblique
tendon near the trochlea or both! I now recommend
transfer of the superior oblique tendon without
attempting to fracture the trochlea. While this is not B
a perfect procedure, nothing that I know of can be
expected to yield a perfect result in cases of third
nerve palsy.
The procedure begins with a large limbal inci-
sion exposing the adjacent corners of the medial and
superior rectus muscles (Figure 7). The superior
oblique tendon is seen nasal to the superior rectus
muscle as it passes in Tenons capsule toward its inser-
tion. The tendon is engaged on a small hook and is
shelled out of Tenon's as described in chapter 9. The
tendon is pulled forward and is cut as near the inser-
tion as possible in order to have sufficient tendon
C
length. A small clamp should be placed on the supe-
rior oblique tendon before the tendon is transected.
The tendon is